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1.  Shone’s anomaly: a report of one case in sub-Saharan Africa 
Shone’s anomaly is a rare congenital cardiac malformation consisting of four obstructive lesions of the left heart and the aortic arch: a supramitral membrane; parachute mitral valve; subaortic stenosis; and coarctation of the aorta which occurs most commonly in its incomplete form. We report on a 17-year-old male Cameroonian with a variant of shone anomaly consisting of sub-valvular aortic stenosis, coarctation of the aorta and parachute mitral valve. He was referred for the investigation of a cardiac murmur after he presented with shortness of breath preceded by flu like syndrome. He underwent successful management abroad consisting of aortic coarctation treatment by percutaneous angioplasty and resection of the subaortic ring by traditional surgical approach without any mitral valve repair. Shone’s anomaly is a rare congenital heart disease that can be missed in low resource settings. This case raises awareness about this syndrome, highlights the importance of cardiac ultrasonography in resource limited settings and illustrates the need for comprehensive service frameworks to improve access and level of care and services for patients in the African context.
doi:10.3978/j.issn.2223-3652.2014.12.04
PMCID: PMC4278041  PMID: 25610807
Congenital heart disease; Shone’s anomaly; Cameroon; Sub-Saharan Africa (SSA)
2.  Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960-91): a study of 313 patients. 
British Heart Journal  1994;71(6):588-595.
OBJECTIVE--To determine the incidence of the various types of obstruction of the left ventricular outflow tract in patients born in the five health districts of Liverpool and to compare their prognosis into early adult life. DESIGN--Notes of all patients with obstruction of the left ventricular outflow tract born in the study area between 1960 and 1991 were reviewed. Patients with hypoplastic left ventricle, mitral valve atresia, and those with discordant atrioventricular or ventriculoarterial connections were excluded. Survivors were traced and assessed clinically; eight were lost to follow up. RESULTS--Obstruction of the left ventricular outflow tract occurred in 313 patients (67% male), giving an incidence of 6.1/10,000 live births. The median (range) age at presentation was 13.9 months (0-20 yr). Aortic valve stenosis occurred in 71.2%: subvalve in 13.7%, supravalve in 7.7%, and multilevel in 7.4%. The median (range) duration of follow up was 10.0 (1-29) yr. Aortic regurgitation at presentation occurred more often (p < 0.001) in patients with subvalve stenosis than in those with other types of obstruction, but there was an increased incidence (p < 0.001) at follow up in patients with valve stenosis. Ninety eight patients (31.3%) underwent operation. The reoperation rate was 27% for valve stenosis and 9% for subvalve obstruction. No patients with supravalve stenosis underwent reoperation. The median duration from first operation to aortic valve replacement (17 patients) was 12.3 years. Hazard analysis confirmed that the risk of death was higher in patients presenting at a younger age, with more severe stenosis, and those with subaortic, multilevel obstruction or a syndrome. Hazard analysis also showed that the risk of a clinical event (surgery, balloon dilatation, or endocarditis) was greater in patients who presented at a younger age, with more severe stenosis or aortic regurgitation, and in those with subvalve or multilevel obstruction. CONCLUSIONS--Aortic valve stenosis was the most common type of obstruction. Hazard analysis indicates that the age and severity of obstruction at presentation have a significant effect on survival and freedom from a clinical event. The risk of premature death in patients presenting with moderately severe valve stenosis is reasonably small, but increases considerably in those with subvalve, supravalve, and multilevel obstruction. Patients who present with mild valve stenosis have a good prognosis. The risk of sudden death is less than previous predictions. Patients with subvalve and multilevel obstruction, even when mild at presentation, are more likely to undergo intervention or develop endocarditis than those with valve or supravalve stenosis. Follow up into adult life is essential.
PMCID: PMC1025460  PMID: 8043345
3.  Surgical outcome of partial Shone complex 
Partial forms of Shone complex are rare. Surgical outcomes of the complete forms have generally been poor, whereas there is a lack of data on long-term follow-up of surgically treated adult partial complex. Between 2001 and 2011, nine patients (age: 38 ± 8 years; six males, 67%) were referred for valvular heart disease. Transthoracic and transoesophageal echocardiography was performed. Data were confirmed by intra-operative findings and reports. Patients were diagnosed as partial Shone complex and presented with mitral stenosis (MS) (45%) or mitral regurgitation (22%) or aortic regurgitation (22%). All but one patient (89%) reported previous surgery: coarctation of the aorta repair (87.5%) and aortic valvulotomy (12.5%). Redo intervention included: mitral valve replacement (25%), mitral repair (25%), aortic valve replacement (37.5%) and subvalvular aortic ridge resection (25%). One patient refused surgery. Patients surgically treated before the age of 5 (87.5%) showed favourable outcome (survival rate: 100%) and a 23.6 (± 4.6)-year follow-up free from events. The patient who underwent first intervention at the age of 50 and the patient with MS who refused surgery showed a 45 (± 7)-year follow-up free from major morbidity. Patients with partial Shone complex, properly diagnosed and treated, show favourable surgical outcome free from major clinical events.
doi:10.1093/icvts/ivr169
PMCID: PMC3309834  PMID: 22279117
Parachute mitral valve; Aortic coarctation; Shone's syndrome; Congenital heart disease
4.  Ross-Konno Operation for Patients with Shone Complex 
Texas Heart Institute Journal  2010;37(2):240-241.
We report the cases of 2 pediatric patients who had Shone complex. Each child had severe left ventricular outflow tract obstruction and marginal mitral obstruction, and both underwent the Ross-Konno operation. The mitral valve was left alone. Both patients survived the operations, and serial follow-up echocardiography up to 4 years later showed a decreased or decreasing trend in the peak mitral diastolic velocity. The Ross-Konno procedure can be an acceptable approach for a subgroup of patients who have Shone complex.
PMCID: PMC2851429  PMID: 20401305
Aortic valve stenosis/congenital/surgery; cardiac surgical procedures/methods; child, preschool; heart defects, congenital/mortality/pathology/surgery; mitral valve/abnormalities/pathology/surgery; reoperation; risk assessment; treatment outcome; ventricular outflow obstruction/complications/congenital/surgery
5.  Left Ventricular Assist Devices 
Executive Summary
Objective
The objective of this health technology policy assessment was to determine the effectiveness and cost-effectiveness of using implantable ventricular assist devices in the treatment of end-stage heart failure.
Heart Failure
Heart failure is a complex syndrome that impairs the ability of the heart to maintain adequate blood circulation, resulting in multiorgan abnormalities and, eventually, death. In the period of 1994 to 1997, 38,702 individuals in Ontario had a first hospital admission for heart failure. Despite reported improvement in survival, the five-year mortality rate for heart failure is about 50%.
For patients with end-stage heart failure that does not respond to medical therapy, surgical treatment or traditional circulatory assist devices, heart transplantation (in appropriate patients) is the only treatment that provides significant patient benefit.
Heart Transplant in Ontario
With a shortage in the supply of donor hearts, patients are waiting longer for a heart transplant and may die before a donor heart is available. From 1999 to 2003, 55 to 74 people received a heart transplant in Ontario each year. Another 12 to 21 people died while waiting for a suitable donor heart. Of these, 1 to 5 deaths occurred in people under 18 years old. The rate-limiting factor in heart transplant is the supply of donor hearts. Without an increase in available donor hearts, attempts at prolonging the life of some patients on the transplant wait list could have a harmful effect on other patients that are being pushed down the waiting list (knock on effect).
LVAD Technology
Ventricular assist devices [VADs] have been developed to provide circulatory assistance to patients with end-stage heart failure. These are small pumps that usually assist the damaged left ventricle [LVADs] and may be situated within the body (intracorporeal] or outside the body [extracorporeal). Some of these devices were designed for use in the right ventricle [RVAD] or both ventricles (bi-ventricular).
LVADs have been mainly used as a “bridge-to-transplant” for patients on a transplant waiting list. As well, they have been used as a “bridge-to-recovery” in acute heart failure, but this experience is limited. There has been an increasing interest in using LVAD as a permanent (destination) therapy.
Review of LVAD by the Medical Advisory Secretariat
The Medical Advisory Secretariat’s review included a descriptive synthesis of findings from five systematic reviews and 60 reports published between January 2000 and December 2003. Additional information was obtained through consultation and by searching the websites of Health Canada, the United Network of Organ Sharing, Organ Donation Ontario, and LVAD manufacturers.
Summary of Findings
Safety and Effectiveness
Previous HTAs and current Level 3 evidence from prospective non-randomized controlled studies showed that when compared to optimal medical therapy, LVAD support significantly improved the pre-transplant survival rates of heart transplant candidates waiting for a suitable donor heart (71% for LVAD and 36% for medical therapy). Pre-transplant survival rates reported ranged from 58% to 90% (median 74%). Improved transplant rates were also reported for people who received pre-transplant LVAD support (e.g. 67% for LVAD vs 33% for medical therapy). Reported transplant rates for LVAD patients ranged from 39% to 90% (median 71%).
Patient’s age greater than 60 years and pre-existing conditions of respiratory failure associated with septicemia, ventilation, and right heart failure were independent risk factors for mortality after the LVAD implantation.
LVAD support was shown to improve the New York Heart Association [NYHA)] functional classification and quality of life of patients waiting for heart transplant. LVAD also enabled approximately 41% - 49% of patients to be discharged from hospitals and wait for a heart transplant at home. However, over 50% of the discharged patients required re-hospitalization due to adverse events.
Post-transplant survival rates for LVAD-bridged patients were similar to or better than the survival rates of patients bridged by medical therapy.
LVAD support has been associated with serious adverse events, including infection (median 53%, range 6%–72%), bleeding (8.6%–48%, median 35%), thromboembolism (5%–37%), neurologic disorders (7%–28%), right ventricular failure (11%–26%), organ dysfunction (5%–50%) and hemolysis (6%–20%). Bleeding tends to occur in the first few post-implant days and is rare thereafter. It is fatal in 2%–7% of patients. Infection and thromboembolism occurred throughout the duration of the implant, though their frequency tended to diminish with time. Device malfunction has been identified as one of the major complications. Fatalities directly attributable to the devices were about 1% in short-term LVAD use. However, mechanical failure was the second most frequent cause of death in patients on prolonged LVAD support. Malfunctions were mainly associated with the external components, and often could be replaced by backed up components.
LVAD has been used as a bridge-to-recovery in patients suffering from acute cardiogenic shock due to cardiomyopathy, myocarditis or cardiotomy. The survival rates were reported to be lower than in bridge-to-transplant (median 26%). Some of the bridge-to-recovery patients (14%–75%) required a heart transplant or remained on prolonged LVAD support. According to an expert in the field, experience with LVAD as a bridge-to-recovery technology has been more favourable in Germany than in North America, where it is not regarded as a major indication since evidence for its effectiveness in this setting is limited.
LVAD has also been explored as a destination therapy. A small, randomized, controlled trial (level 2 evidence) showed that LVAD significantly increased the 1-year survival rate of patients with end-stage heart failure but were not eligible for a heart transplant (51% LVAD vs 25% for medical therapy). However, improved survival was associated with adverse events 2.35 times higher than medically treated patients and a higher hospital re-admission rate. The 2-year survival rate on LVAD decreased to 23%, although it was still significantly better compared to patients on medical therapy (8%). The leading causes of deaths were sepsis (41%) and device failure (17%).
The FDA has given conditional approval for the permanent use of HeartMate SNAP VE LVAS in patients with end-stage heart failure who are not eligible for heart transplantation, although the long-term effect of this application is not known.
In Canada, four LVAD systems have been licensed for bridge-to-transplant only. The use of LVAD support raises ethical issues because of the implications of potential explantation that could be perceived as a withdrawal of life support.
Potential Impact on the Transplant Waiting List
With the shortage of donor hearts for adults, LVAD support probably would not increase the number of patients who receive a heart transplant. If LVAD supported candidates are prioritized for urgent heart transplant, there will be a knock on effect as other transplant candidates without LVAD support would be pushed down, resulting in longer wait, deterioration in health status and die before a suitable donor heart becomes available.
Under the current policy for allocating donor hearts in Ontario, patients on LVAD support would be downgraded to Status 3 with a lower priority to receive a transplant. This would likely result in an expansion of the transplant waiting list with an increasing number of patients on prolonged LVAD support, which is not consistent with the indication of LVAD use approved by Health Canada.
There is indication in the United Kingdom that LVAD support in conjunction with an urgent transplant listing in the pediatric population may decrease the number of deaths on the waiting list without a harmful knock-on effect on other transplant candidates.
Conclusion
LVAD support as a bridge-to-transplant has been shown to improve the survival rate, functional status and quality of life of patients on the heart transplant waiting list. However, due to the shortage of donor hearts and the current heart transplant algorithm, LVAD support for transplant candidates of all age groups would likely result in an expansion of the waiting list and prolonged use of LVAD with significant budget implications but without increasing the number of heart transplants. Limited level 4 evidence showed that LVAD support in children yielded survival rates comparable to those in the adult population. The introduction of LVAD in the pediatric population would be more cost-effective and might not have a negative effect on the transplant waiting list.
PMCID: PMC3387736  PMID: 23074453
6.  Morphology of left ventricular outflow tract structures in patients with subaortic stenosis and a ventricular septal defect. 
British Heart Journal  1994;72(3):251-260.
OBJECTIVE--To compare the incidence and prognosis of subaortic stenosis associated with a ventricular septal defect and to define the morphological basis of subaortic stenosis. DESIGN--Presentation and follow up data on 202 patients with subaortic stenosis seen at the Royal Liverpool Children's Hospital between 1 January 1960 and 31 December 1991 were reviewed. Survivors were traced to assess their current clinical state. Necropsy specimens of 291 patients with lesions associated with subaortic stenosis were also examined. RESULTS--In the clinical study; 65 (32.1%) of the 202 patients with subaortic stenosis had a ventricular septal defect (excluding an atrioventricular septal defect). 32 of these patients had a short segment (fibromuscular) subaortic stenosis. 33 had subaortic stenosis produced by deviation of muscular components of the outflow tracts. In 17 patients (51.5%) this was caused by posterior deviation or extension of structures into the left ventricular outflow tract, resulting in obstruction above the ventricular septal defect. In the other 16 patients (48.5%) there was over-riding of the aorta with concordant ventriculoarterial connections, (without compromise to right ventricular outflow) producing subaortic stenosis below the ventricular septal defect. Additional fibrous obstruction occurred in 39% of the patients with deviated structures. The age at presentation was lower (P < 0.01) in patients with deviated structures (median (range) 0.4 (0 to 9.2) months) than in those with short segment obstruction (median (range) 4.2 (0 to 84.9) months). The incidence of aortic arch obstruction was higher (P < 0.002) in patients with deviated structures than in those with short segment obstruction (38%). In the morphological study 35 pathological specimens showed obstructive muscular structures in the left ventricular outflow tract either above or below the ventricular septal defect. 16 had either posterior deviation of the outlet septum or extension of the right ventriculoinfundibular fold, or both of these together into the left ventricle. 19 had anterior deviation of the outlet septum into the right ventricle with overriding of the aorta (without compromise to right ventricular outflow). The earliest age at which additional fibrous obstruction was seen was 9 months. The aortic valve circumference was small in 18% of specimens. FOLLOW UP--The median (range) duration of follow up in survivors from the clinical study was 6.6 (1 to 25.7) years. 16 patients with deviated musculature (49%) and 16 with short segment fibromuscular stenosis (50%) underwent operation for subaortic stenosis. Patients with deviated structures were younger at operation than those with short segment stenosis (P < 0.005). Patients with posterior deviation or extension of structures into the left ventricular outflow tract underwent operation for subaortic stenosis more frequently (P < 0.05) than those with anterior deviation of the outlet septum and aortic override. The ventricular septal defect required surgical closure more frequently (P < 0.005) in patients with deviation (93.9%) than in those with short segment obstruction (21.9%). There was no significant difference in the mortality between patients with deviation (27%) and those with short segment obstruction (12%). CONCLUSIONS--32% of patients in the clinical study with subaortic stenosis had a ventricular septal defect. Only 51% of these had obstructive and deviated muscular structures in the left ventricular outflow tract. These patients had a significantly higher incidence of aortic arch obstruction and required surgery for subaortic stenosis at a younger age than those with short segment obstruction. The ventricular septal defect also required surgical closure more frequently in those patients with deviation. The morphological study defined the two sites of obstruction. The presence or absence and type of deviation should be clearly defined in all patients with a ventricular septal defect,
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PMCID: PMC1025511  PMID: 7946776
7.  Role of connexins in human congenital heart disease: the chicken and egg problem 
Inborn cardiac diseases are among the most frequent congenital anomalies and are the main cause of death in infants within the first year of age in industrialized countries when not adequately treated. They can be divided into simple and complex cardiac malformations. The former ones, for instance atrial and ventricular septal defects, valvular or subvalvular stenosis or insufficiency account for up to 80% of cardiac abnormalities. The latter ones, for example transposition of the great vessels, Tetralogy of Fallot or Shone’s anomaly often do not involve only the heart, but also the great vessels and although occurring less frequently, these severe cardiac malformations will become symptomatic within the first months of age and have a high risk of mortality if the patients remain untreated. In the last decade, there is increasing evidence that cardiac gap junction proteins, the connexins (Cx), might have an impact on cardiac anomalies. In the heart, mainly three of them (Cx40, Cx43, and Cx45) are differentially expressed with regard to temporal organogenesis and to their spatial distribution in the heart. These proteins, forming gap junction channels, are most important for a normal electrical conduction and coordinated synchronous heart muscle contraction and also for the normal embryonic development of the heart. Animal and also some human studies revealed that at least in some cardiac malformations alterations in certain gap junction proteins are present but until today no particular gap junction mutation could be assigned to a specific cardiac anomaly. As gap junctions have often been supposed to transmit growth and differentiation signals from cell to cell it is reasonable to assume that they are somehow involved in misdirected growth present in many inborn heart diseases playing a primary or contributory role. This review addresses the potentional role of gap junctions in the development of inborn heart anomalies like the conotruncal heart defects.
doi:10.3389/fphar.2013.00070
PMCID: PMC3669755  PMID: 23760510
connexin; gap junction; cardiac malformations; cardiac morphogenesis; mutation
8.  Supravalvar mitral stenosis: risk factors for recurrence or death after resection. 
British Heart Journal  1995;73(2):164-168.
OBJECTIVE--To assess the medium term outcome in infants and children after surgical resection of supravalvar mitral stenosis with special reference to risk factors for mortality or recurrence of supravalvar mitral stenosis. No detailed follow up has been previously reported in this uncommon condition. DESIGN--Prospective cross sectional clinical and echocardiographic follow up. SETTING--Paediatric cardiothoracic unit. PATIENTS AND METHODS--23 consecutive children (14 male, nine female, mean age 3 years 2 months at surgery) who underwent resection of supravalvar mitral stenosis between 1978 and 1993. RESULTS--Follow up was for a mean of 58 months (range 0.5-167) after resection of supravalvar mitral stenosis. Four patients developed recurrent supravalvar mitral stenosis: this has not been reported previously. This was recognised 14-108 months after resection and confirmed at repeat operation. Three of these patients had successful reoperations but one died. Five other patients died. On multivariate analysis the only variable associated with survival free of recurrent supravalvar mitral stenosis was older age (18 months or more) at time of surgery (hazard ratio 0.17, 95% confidence interval (CI) 0.03 to 0.95, P < 0.05). Five year actuarial survival free of recurrent obstruction when supravalvar mitral stenosis was resected at age less than 18 months was only 39% (95% CI 9 to 69%) compared with 73% (95% CI 24 to 93%) in older patients. CONCLUSION--Supravalvar mitral stenosis is part of a spectrum of obstructive lesions affecting the left heart. Recurrent supravalvar mitral stenosis can develop after surgical resection. The prognosis in those who require resection within the first 18 months of life is poor: mortality is high, as is the risk of recurrent supravalvar mitral stenosis in survivors, probably because of continuing turbulent flow across a small left ventricular inflow tract.
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PMCID: PMC483784  PMID: 7696027
9.  Natural history and surgical outcomes for isolated discrete subaortic stenosis in children 
Heart  1999;82(6):708-713.
OBJECTIVE—To document the natural history and surgical outcomes for discrete subaortic stenosis in children.
DESIGN—Retrospective review.
SETTING—Tertiary care paediatric cardiology centres.
PATIENTS—92 children diagnosed between 1985 and 1998.
MAIN OUTCOME MEASURES—Echocardiographic left ventricular outflow gradient (echograd), and aortic insufficiency (AI).
RESULTS—The mean (SEM) age at diagnosis was 5.3 (0.4) years; the mean echograd was 30 (2) mm Hg, with AI in 22% (19/87) of patients. The echograd and incidence of AI increased to 35 (3) mm Hg and 53% (36/68) (p < 0.05) 3.6 (0.3) years later. The echograd at diagnosis predicted echograd progression and appearance of AI. 42 patients underwent surgery 2.2 (0.4) years after diagnosis. Preoperatively echograd and AI incidence increased to 58 (6) mm Hg and 76% (19/25) (p < 0.05). The echograd was 26 (4) mm Hg 3.7 (0.4) years postoperatively, with AI in 82% (31/38) of patients. Surgical morbidities included complete heart block, need for prosthetic valves, and iatrogenic ventricular septal defects. Eight patients underwent reoperation for recurrent subaortic stenosis. The age at diagnosis of 44 patients followed medically and 42 patients operated on did not differ (5.5 (0.6) v 5.0 (0.6) years, p < 0.05). However, the echograd at diagnosis in the former was less (21 (2) v 40 (5) mm Hg, p < 0.05) and did not increase (23 (2) mm Hg) despite longer follow up (4.1 (0.4) v 2.2 (0.4) years, p < 0.05). The incidence of AI at diagnosis and at last medical follow up was also less (14% (6/44) v 34% (13/38); 40% (17/43) v 76% (19/25), p < 0.05).
CONCLUSIONS—Many children with mild subaortic stenosis exhibit little progression of obstruction or AI and need not undergo immediate surgery. Others with more severe subaortic stenosis may progress precipitously and will benefit from early resection despite risks of surgical morbidity and recurrence.


Keywords: subaortic stenosis; congenital heart disease; cardiac surgery
PMCID: PMC1729222  PMID: 10573499
10.  Persistent fifth aortic arch diagnosed by echocardiography and confirmed by angiography: Case report and literature review 
Persistent fifth aortic arch is a rare congenital anomaly that can be discovered incidentally or at postmortem exam. It can be associated with major congenital heart malformations involving the systemic or the pulmonary circuits. It usually has no clinical significance but can be either, beneficial as in systemic outflow tract obstructions or cause hemodynamic compromise when associated with a significant left to right shunt. We report an infant with persistent fifth aortic arch associated with Shone’s complex diagnosed accurately by transthoracic echocardiography and confirmed by cardiac catheterization and computed tomography.
doi:10.1016/j.jsha.2009.10.003
PMCID: PMC3727347  PMID: 23960580
Persistent fifth aortic arch; Shone complex; Coarctation of the aorta
11.  An Unusual Presentation of Congenital Heart Disease 
Texas Heart Institute Journal  2003;30(3):214-217.
We describe an unusual presentation of congenital heart disease mimicking preeclampsia in a young, gravid girl. The diagnosis of Shone's complex was confirmed by echocardiography. This complex is manifested by multiple levels of obstruction involving the left side of the heart and the systemic circulation. It is extremely rare, especially in pregnant adolescents. We briefly describe the patient's clinical history, physical examination, and treatment, as well as our clinical decisions regarding her case. The discussion focuses on the spectrum of findings in Shone's complex and the physiologic impact of therapy on this patient. (Tex Heart Inst J 2003;30:214–7)
PMCID: PMC197320  PMID: 12959205
Heart disease, congenital; mitral valve stenosis; pregnancy; Shone's complex
12.  Muscular (hypertrophic) subaortic stenosis (hypertrophic obstructive cardiomyopathy): the evidence for true obstruction to left ventricular outflow. 
Postgraduate Medical Journal  1986;62(728):531-536.
The clinical and haemodynamic significance of the subaortic pressure gradient in patients with muscular (hypertrophic) subaortic stenosis (hypertrophic obstructive cardiomyopathy) has long been debated. In this report we summarize the evidence which indicates that true obstruction to left ventricular outflow exists in these patients. Rapid left ventricular ejection, through an outflow tract narrowed by ventricular septal hypertrophy, results in Venturi forces causing systolic anterior motion of the anterior (or posterior) mitral leaflets. Mitral leaflet-septal contact results in obstruction to outflow and the accompanying mitral regurgitation. The time of onset of mitral leaflet-septal contact determines the magnitude of the pressure gradient and the severity of the mitral regurgitation, as well as the degree of prolongation of left ventricular ejection time and the percentage of left ventricular stroke volume that is ejected in the presence of an obstructive pressure gradient. Early and prolonged mitral leaflet-septal contact results in a large pressure gradient, significant mitral regurgitation, as well as dramatic prolongation of the ejection time and a large percentage of left ventricular stroke volume being obstructed. Late and short mitral leaflet-septal contact results in little haemodynamic perturbation. Hypertrophic cardiomyopathy patients with obstructive pressure gradients are significantly more symptomatic than those without. Thus the obstructive pressure gradients in hypertrophic cardiomyopathy are of clinical as well as haemodynamic significance. To deny the existence of obstruction to outflow in patients with muscular subaortic stenosis is to deny these patients appropriate medical and surgical therapy.
PMCID: PMC2418801  PMID: 3774688
13.  Primary Left Ventricular Rehabilitation is Effective in Maintaining Two-Ventricle Physiology in the Borderline Left Heart 
Objective
Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenosis, left ventricular (LV) hypoplasia) and endocardial fibroelastosis (EFE). The multilevel obstruction and impaired LV systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of LV rehabilitation - EFE resection with mitral and/or aortic valvuloplasty - on LV function and clinical outcomes.
Methods
All patients with borderline left heart structures and EFE who underwent primary LV rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from pre- and post-operative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after LV rehabilitation. Pre- and post-operative values were compared by paired t-test.
Results
Between 1999 and 2008, 9 patients with EFE and borderline left heart underwent LV rehabilitation at a median age of 5.6 months (range 1–38 months). There was no operative mortality, and at a median follow up of 25 months (6 months to 10 years), there was one death from non cardiac causes, and two patients required reoperations. Significant increase in ejection fraction and LV end diastolic volume were observed, whereas left atrial pressure and RV/LV pressure ratios decreased postoperatively.
Conclusion
In patients with borderline left hearts, primary LV rehabilitation with EFE resection and mitral and aortic valvuloplasty results in improved LV systolic and diastolic performance and decreased RV pressures. This approach may provide an alternative to single ventricle management in this difficult patient group.
doi:10.1016/j.jtcvs.2009.08.009
PMCID: PMC2787646  PMID: 19819471
14.  Fixed subaortic stenosis: anatomical spectrum and nature of progression. 
British Heart Journal  1991;65(5):280-286.
Retrospective echocardiographic review identified 58 consecutive infants and children with fixed subaortic stenosis. Mean (SD) age at diagnosis was 4.8 (3.6) years (range two days to 14.7 years), and diagnosis occurred in infancy in eight. Associated cardiac abnormalities were present in 41 (71%) whereas fixed subaortic stenosis was an isolated lesion in 17 (29%). Four types of fixed subaortic stenosis were identified: short segment (47 (81%)), long segment (7 (12%)), posterior displacement of the infundibular septum with additional discrete narrowing of the left ventricular outflow tract (3 (5%)), and redundant tissue arising from the membranous septum (1 (2%)). Echocardiographic studies had been performed before the diagnosis of fixed subaortic stenosis in nine patients, all with associated abnormalities. These were performed in infancy in each and showed a "normal" left ventricular outflow tract in six and posterior deviation of the infundibular septum in three. In 16 patients serial echocardiographic studies had been performed after the diagnosis of fixed subaortic stenosis but before surgery of the left ventricular outflow tract. Rapid evolution of short segment to long segment narrowing was seen in one patient, and tethering of the aortic valve or mitral valve developed in a further four patients. Aortic valve or mitral valve involvement was not seen before the age of three years but was common thereafter (10/40 patients, 25%). Fixed subaortic stenosis may be an "acquired" lesion with the potential for changes in form as well as progression in severity of left ventricular outflow tract obstruction.
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PMCID: PMC1024631  PMID: 2039674
15.  Juvenile severe mitral stenosis predisposing Eisenmenger syndrome in a case with ventricular septal defect, patent ductus arteriosus, coarctation of aorta & hypoplastic aortic arch: Report of first case of rare association 
We are reporting the first case of rare association between multiple congenital cardiac malformations with severe rheumatic mitral stenosis which is an acquired structural cardiac disease. A 16 years old female patient presented with progressive dyspnoea & cyanosis for the last one month with past history of recurrent pneumonia since infancy. Physical examination revealed presence of cyanosis, grade I clubbing, radio-radial & radio-femoral delay, loud & single second heart sound, apical long mid diastolic murmur and left parasternal ejection systolic murmur. Transthoracic echocardiography revealed severe rheumatic mitral stenosis, multiple ventricular septal defects (VSD) with bidirectional shunt, hypoplastic aortic arch, Coarctation of aorta and severe pulmonary hypertension. Transesophageal echocardiography revealed the same findings along with the presence of moderate mitral regurgitation and 9 mm perimembranous VSD extending into muscular septum. Cardiac catheterization study confirmed the echocardiographic findings and demonstrated large patent ductus arteriosus (PDA). We have planned for high-risk percutaneous transmitral commissurotomy (PTMC) for this patient to decrease the back pressure on pulmonary vasculature. So that right to left shunt will be decreased and cyanosis will also improve. But parents refused to give consent for PTMC. She was on treatment with regular penicillin prophylaxis, diuretics, sildenafil and infective endocarditic prophylaxis. We should be aware of this kind of complex association between congenital and acquired structure heart disease. Eisenmenger syndrome could also be a presentation of juvenile severe rheumatic mitral stenosis when it is associated with congenital shunt lesion like VSD/PDA in our case.
doi:10.1016/j.jcdr.2013.05.006
PMCID: PMC3849316  PMID: 24396261
Juvenile mitral stenosis; Eisenmenger syndrome; Ventricular septal defects; Hypoplastic aortic arch; Patent ductus arteriosus
16.  Nongenetically transmitted disproportionate ventricular septal thickening associated with left ventricular outflow obstruction. 
British Heart Journal  1979;41(3):345-349.
Clinical, haemodynamic, and morphological features are described in 2 patients with disproportionate ventricular septal thickening, left ventricular outflow obstruction with systolic anterior motion of the anterior mitral leaflet, and either acquired or congenital heart disease. The disproportionate septal thickening in these patients appeared to be secondary to their underlying cardiac disease rather than a manifestation of genetically transmitted hypertrophic cardiomyopathy. One patient with combined aortic and mitral stenosis had severe systolic anterior motion of the anterior mitral leaflet and a residual large systolic pressure gradient between left ventricle and systemic artery after aortic valve replacement. In this patient the systolic anterior motion was evident in the presence of mitral valve stenosis. The other patient with mild aortic stenosis and a previously repaired coarctation of the aorta also had mild systolic anterior motion and a small subaortic systolic pressure gradient. Hence, these 2 patients demonstrate that disproportionate septal thickening secondary to acquired or congenital heart disease may be associated with left ventricular outflow obstruction and systolic anterior motion of the anterior mitral leaflet.
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PMCID: PMC482038  PMID: 154915
17.  Surgical Management of Left Ventricular Outflow Tract Obstruction after Biventricular Repair of Double Outlet Right Ventricle 
Journal of Korean Medical Science  2010;25(3):374-379.
Regardless of the preoperative morphology and the type of operation, left ventricular outflow tract obstruction (LVOTO) after biventricular repair of double outlet right ventricle (DORV) may develop. This report presents our 10-yr experience with surgical management of LVOTO after biventricular repair of DORV. Between 1996 and 2006, 15 patients underwent reoperation for subaortic stenosis after biventricular repair of DORV. The mean age at biventricular repair was 23.3±18.3 months (1.1-64.2). Biventricular repairs included tunnel constructions from the left ventricle to the aorta in 14 cases and an arterial switch operation in one. The mean left ventricle-to-aorta peak pressure gradient was 54.0±37.7 mmHg (15-140) after a mean follow-up of 9.5±6.3 yr. We performed extended septoplasty in nine patients and fibromuscular resection in six. There were no early or late mortality. There was one heart block and one aortic valve injury after an extended septoplasty, and two and one after a fibromuscular resection. No patient required reoperation for recurrent subaortic stenosis. The mean pressure gradient was 11.2±11.4 mmHg (0-34) after a mean follow-up of 5.6±2.7 yr. Extended septoplasty is a safe and effective method for the treatment of subaortic stenosis, especially in cases with a long-tunnel shaped LVOTO.
doi:10.3346/jkms.2010.25.3.374
PMCID: PMC2826730  PMID: 20191035
DORV; Aortic Stenosis, Subvalvular
18.  Discrete Subaortic Stenosis 
Texas Heart Institute Journal  2003;30(4):286-292.
Discrete subaortic stenosis, which is an obstructing lesion of the left ventricular outflow tract, remains a surgical challenge. The recurrence rate is high despite sufficient conventional resection.
We retrospectively reviewed the results of surgery for discrete subaortic stenosis at our institution from September 1995 through March 2001. Twenty-one patients with this lesion underwent surgical treatment during this period. Excision of the fibromuscular membrane with myectomy was performed in all of the patients. Follow-up in all patients ranged from 7 to 67 months (mean follow-up period, 39.57 ± 15.46 months).
The mean systolic gradient between the left ventricle and the aorta decreased from 59.23 ± 35.38 mmHg preoperatively to 9.47 ± 9.91 mmHg postoperatively. There was no instance of heart block that required a permanent pacemaker, nor of bacterial endocarditis. There was no early or late postoperative death. A 22nd patient, who had 3+ aortic regurgitation, required aortic valve replacement and was excluded from the study. Two of the patients (9.5%) underwent reoperation because of recurrent gradient and residual ventricular septal defect.
Our results suggest that fibromuscular membrane excision combined with myectomy in patients with discrete subaortic stenosis produces sufficient relief of obstruction with low morbidity. (Tex Heart Inst J 2003;30:286–92)
PMCID: PMC307713  PMID: 14677738
Aortic valve stenosis/surgery; cardiac surgical procedures; heart septal defects, ventricular; myectomy; retrospective studies; ventricular outflow obstruction/surgery
19.  Exuberant accessory mitral valve tissue with possible true parachute mitral valve: a case report 
Introduction
A parachute mitral valve is defined as a unifocal attachment of mitral valve chordae tendineae independent of the number of papillary muscles. Data from the literature suggests that the valve can be distinguished on the basis of morphological features as either a parachute-like asymmetrical mitral valve or a true parachute mitral valve. A parachute-like asymmetrical mitral valve has two papillary muscles; one is elongated and located higher in the left ventricle. A true parachute mitral valve has a single papillary muscle that receives all chordae, as was present in our patient. Patients with parachute mitral valves during childhood have multilevel left-side heart obstructions, with poor outcomes without operative treatment. The finding of a parachute mitral valve in an adult patient is extremely rare, especially as an isolated lesion. In adults, the unifocal attachment of the chordae results in a slightly restricted valve opening and, more frequently, valvular regurgitation.
Case presentation
A 40-year-old Caucasian female patient was admitted to a primary care physician due to her recent symptoms of heart palpitation and chest discomfort on effort. Transthoracic echocardiography showed chordae tendineae which were elongated and formed an unusual net shape penetrating into left ventricle cavity. The parasternal short axis view of her left ventricle showed a single papillary muscle positioned on one side in the posteromedial commissure receiving all chordae. Her mitral valve orifice was slightly eccentric and the chordae were converting into a single papillary muscle. Mitral regurgitation was present and it was graded as moderate to severe. Her left atrium was enlarged. There were no signs of mitral stenosis or a subvalvular ring. She did not have a bicuspid aortic valve or coarctation of the ascending aorta. The dimensions and systolic function of her left ventricle were normal. Our patient had a normal body habitus, without signs of heart failure. Her functional status was graded as class I according to the New York Heart Association grading.
Conclusions
A recently published review found that, in the last several decades, there have been only nine adult patients with parachute mitral valve disease reported, of which five had the same morphological characteristics as our patient. This case presentation should encourage doctors, especially those involved in echocardiography, to contribute their own experience, knowledge and research in parachute mitral valve disease to enrich statistical and epidemiologic databases and aid clinicians in getting acquainted with this rare disease.
doi:10.1186/1752-1947-6-292
PMCID: PMC3459793  PMID: 22967395
20.  B-scan ultrasonography in idiopathic hypertrophic subaortic stenosis. Study of left ventricular outflow tract and mechanism of obstruction. 
British Heart Journal  1976;38(6):595-604.
Studies were made with standard time motion and B-scan echocardiography on 48 patients including 5 with idiopathic hypertrophic subaortic stenosis (hypertrophic obstructive cardiomyopathy), undergoing diagnostic cardiac catheterization. The dimensions of the left ventricular outflow (O) and inflow (I) tracts were measured on the B-scan images. The outflow tract was significantly narrowed in idiopathic hypertrophic subaortic stenosis at both end-systole (1-1+/-0-1 cm) and end-diastole (1-3+/-0-1 cm) when compared with the average width in other patients (2-6+/-0-1 and 3-0+/-0-1 cm, at end-systole and end-diastole, respectively) (P less than 0-001) or normal subjects (2-4+/-0-3 and 2-9+/-0-2 cm) (P less than 0-01). Furthermore, the O/I ratio differed significantly in idiopathic hypertrophic subaortic stenosis (0-5+/-0-1 at end-systole and 0-6+/-0-1 at end-diastole) from that in all other groups (1-4+/-0-1 at both end-systole and end-diastole) (P less than 0-005). There was no appreciable change in the width of the outflow tract from mid- to end-systole in the two patients in whom this was examined. The data support the contention that the anterior leaflet of the mitral valve assumes an abnormally anterior position in idiopathic hypertrophic subaortic stenosis. Though the systolic anterior movement of the tip of the anterior leaflet of the mitral valve shown by M-mode echocardiography could not readily be confirmed with B-scans, we believe that the narrowed outflow tract found in the present investigation contributes to the obstruction that occurs in this disease. We suggest that this outflow tract narrowing is probably caused by hypertrophy of the ventricular septum which in itself contributes to the narrowing, but which also displaces the papillary muscles and thus produces abnormal traction on the mitral valve and striking anterior displacement of the valve apparatus.
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PMCID: PMC483042  PMID: 945063
21.  Echocardiography in combined discrete and hypertrophic subaortic stenosis. 
Thorax  1981;36(2):126-129.
A 10-year-old boy with discrete subaortic stenosis had coexisting abnormal systolic anterior motion of the mitral valve, demonstrated by echocardiography, a sign normally taken as indicating the presence of idiopathic hypertrophic subaortic stenosis. Surgical removal of a fibromuscular diaphragm abolished the echocardiographic signs of discrete subaortic stenosis but abnormal systolic anterior motion of the mitral valve persisted. A severe low cardiac output state complicated immediate recovery after removal of the left ventricle outflow obstruction, and was overcome only with considerable difficulty. The presence of hypertrophied septal muscle, and the associated small left ventricular cavity size, was thought to be the immediate cause of these problems, so that recognition of marked septal hypertrophy, together with abnormal anterior systolic movement of the mitral valve, should serve as a warning that similar difficulties are likely to bae encountered by other patients, after removal of the obstruction in subaortic stenosis. In our experience other forms of left ventricle outflow tract obstruction have not been found to show such a marked degree of asymmetric septal hypertrophy, but this does not mean it may not occur.
PMCID: PMC471455  PMID: 7196603
22.  Prognosis of supravalve aortic stenosis in 81 patients in Liverpool (1960-1993). 
Heart  1996;75(4):396-402.
OBJECTIVE: To determine the prognosis of supravalve aortic stenosis into early adult life and the factors affecting this prognosis. DESIGN: 81 patients with supravalve aortic stenosis were followed for a median duration of 8.3 (range 1 to 29) years. PATIENTS: 40 patients (49.4%) had Williams' syndrome, 18 (22.2%) familial supravalve aortic stenosis, 18 (22.2%) sporadic supravalve aortic stenosis, and five (6.2%) other syndromes. Nineteen patients had additional levels of left ventricular outflow tract obstruction. RESULTS: 47 patients (58%) underwent operation; 20% within a year of presentation. Multivariable analysis predicted that 88% of patients would undergo intervention within 30 years of follow up. The chance of intervention was increased by more severe aortic stenosis at presentation and the presence of multilevel obstruction in patients with sporadic supravalve aortic stenosis. Three deaths occurred before operation and 13 within a month of operation. Ten (62.5%) of the postoperative deaths were in patients with multilevel obstruction. Predicted survival 30 years after presentation was 66%. Risk factors for survival were age and severity of aortic stenosis at presentation. Multilevel obstruction did not emerge as a significant risk factor for death because of the high association with the severity of stenosis at presentation. 74% of survivors had mild or insignificant stenosis at follow up. CONCLUSIONS: Long-term survival is related to age and the severity of aortic stenosis at presentation. Most patients will require intervention, and most survivors will have mild stenosis.
PMCID: PMC484318  PMID: 8705769
23.  A forme fruste of Shone’s anomaly in a 65 year-old patient 
Shone’s anomaly, a congenital cardiac malformation complex, consists of multiple levels of left heart obstruction. A rare case of an incomplete form of this anomaly discovered incidentally during cardiac catheterization for an unrelated event is described.
PMCID: PMC2322915  PMID: 18523519
Congenital heart disease; Shone’s anomaly
24.  Hypoplastic left heart syndrome 
Hypoplastic left heart syndrome(HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch). Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision relative to treatment, and long-term prognosis as information on long-term survival and quality of life for those born with the syndrome is limited.
doi:10.1186/1750-1172-2-23
PMCID: PMC1877799  PMID: 17498282
25.  Fate of patients with fixed subaortic stenosis after surgical removal. 
British Heart Journal  1980;43(6):629-647.
Thirty-nine consecutive patients, aged 5 to 57 years, were followed for two to 15 years with serial haemodynamic studies after removal of fixed subaortic stenosis, which was never a "membrane". Two late deaths occurred, one sudden and one in congestive failure. Of 37 survivors, 25 were asymptomatic and could be classified as good or excellent if judged by well-being. Seven were symptomatic, two having had reoperation for fixed subaortic stenosis, and four needed long-term pacing. Evaluation, including the effect of isoprenaline, showed important dynamic obstruction in 17, five of whom redeveloped fixed obstruction. Seven had congestive features without outflow gradients, and 14 had neither congestion nor outflow obstruction. Complete assessment therefore confirmed that only 14 (36%) were haemodynamically satisfactory; two of them had permanent pacing, and four had had aortic valve surgery. Fixed subaortic stenosis should be removed early, when diagnosed, and completely before secondary myocardial changes occur. Patients however "well" need regular supervision and early haemodynamic assessment. The aortic valve, whether repaired, replaced, or untouched, remains a site for infective endocarditis for life. The fixed subaortic stenosis removed at operation may not be present in that form at birth, but acquired secondary to other congenital abnormalities which remain in the patient.
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PMCID: PMC482764  PMID: 7191709

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