Double-outlet right ventricle (DORV) with a restrictive ventricular septum is a rare but highly morbid phenomenon that can be complicated by progressive left ventricular hypertrophy, arrhythmias, aneurysm formation, severe pulmonary hypertension, and death in the newborn. Surgical creation or enlargement of a ventricular septal defect (VSD) is palliative but may damage the conduction system or the atrioventricular valves in the newborn. This report presents a transcatheter approach to palliation for a newborn that had DORV with a restrictive ventricular septum.
A full-term infant girl (2.9 kg) referred for hypoxia (80 % with room air) and murmur was found to have DORV, interrupted inferior vena cava, and restrictive VSD (95-mmHg gradient). Transhepatic access was performed, and an internal mammary (IM) catheter was advanced through the atrial septal defect and into the left ventricle. By transesophageal echocardiographic guidance, a Baylis radiofrequency perforation wire was used to cross the ventricular septum, and the defect was enlarged using a 4-mm cutting balloon. A bare metal stent then was deployed to maintain the newly created VSD. The patient did well after the procedure but required pulmonary artery banding 4 days later. She returned 5 months later with cyanosis and the development of obstructing right ventricle muscle bundles, requiring further surgical palliation.
This report describes the first transcatheter creation of VSD in DORV with a restrictive ventricular septum in a newborn infant. Use of the radiofrequency catheter in combination with cutting balloon dilation and stent implantation is an efficient method for creating a VSD in such a patient.
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Restrictive VSD; Transcatheter VSD creation; VSD stenting
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.
DORV; Aortic Stenosis, Subvalvular
Total surgical correction of a Taussig-Bing type double outlet right ventricle (DORV) was successfully performed in a severely cyanotic 3-year-old girl. The malformation was associated with bilateral conus, d-transposition of the great arteries, d-loop, and a subpulmonary ventricular septal defect (VSD) without significant pulmonary stenosis in situs solitus. It was impossible to create a tunnel repair by resecting the markedly hypertrophied muscular conus that separated the aortic valve from the subpulmonary ventricular septal defect. Therefore, the VSD was repaired with a Dacron patch, transforming the double outlet right ventricle into a transposition, after which total correction was achieved by means of a Mustard procedure.
Thirty-two patients with double outlet right ventricle (DORV) were studied between 1960 and 1976. Associated congenital defects frequently compounded the difficulty of clinical diagnosis. Cardiac catheterisation was performed in 27 patients, and the ventriculograms were studied with particular regard to the relative positions of the great vessels to each other and to the ventricular septal defect. These relationships determine which corrective operation is possible. Correction has been performed in 12 patients with a perioperative mortality of 25%. Although mitral-aortic discontinuity was demonstrated in all cases, consideration of the anatomical spectrum included in the term DORV suggests that discontinuity is not an essential feature. In common with other clinical data and in contrast with necropsy studies, none of our patients was found to show the normal relationship of the great vessels to each other, in which the aorta lies posterior and to the right of the pulmonary artery. The reason for this difference between the clinical and necropsy findings is not apparent. A similar disparity was shown with regard to pulmonary stenosis, which was demonstrated at catheterisation in 68% of the 27 patients (mean gradient 68 +/- 3 mmHg), in contrast with incidences of 18% and 25% in recent necropsy series. Patients in the necropsy studies were frequently neonates or infants, in whom death may have been the result of intractable cardiac failure secondary to excessive pulmonary blood flow. In older patients without pulmonary stenosis and with pulmonary hypertension, frequent observation is imperative so that surgical treatment can be instituted before the development of irreversible pulmonary vascular disease.
Aortic translocation, although technically demanding, could be an excellent surgical option for d-transposition of the great vessels and left ventricular outflow tract obstruction. We report a modification of the aortic translocation technique that uses autologous tissue. The aortic root is mobilized from the right ventricle with an extension of infundibular free-wall muscle for use in closure of the ventricular septal defect, which is similar to the technique for harvesting pulmonary autograft in the Ross-Konno procedure. Our modification may offer an even better surgical outcome for aortic translocation.
Abnormalities, multiple/surgery; aorta/surgery; cardiac surgical procedures; heart ventricles/surgery; transposition of great vessels/surgery
In three infant cases of double outlet right ventricle (DORV), two with normally related great arteries (NGA) and one with side-by-side great arteries, a transatrial repair was carried out. In all three cases, the results were excellent. It is concluded that in the small baby with DORV with NGA and in DORV with side-by-side great arteries with a hypoplastic crista, a transatrial repair should be successful. This is dependent on the VSD being in the perimembranous (and, therefore, subaortic) location and on the absence of infundibular pulmonary stenosis. In all other varieties of DORV the repair should probably be done through the ventricle.
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.
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
We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction.
Aortic stenosis; Fetal balloon aortic valvuloplasty; Fetal intervention; Hypoplastic left heart syndrome; Resection of endocardial fibroelastosis; Stage 1 hybrid
The basic helix-loop-helix DNA binding protein Hand2 has critical functions in cardiac development both in neural crest-derived and mesoderm-derived structures. Targeted deletion of Hand2 in the neural crest has allowed us to genetically dissect Hand2-dependent defects specifically in outflow tract and cardiac cushion independent of Hand2 functions in mesoderm-derived structures. Targeted deletion of Hand2 in the neural crest results in misalignment of the aortic arch arteries and outflow tract, contributing to development of double outlet right ventricle (DORV) and ventricular septal defects (VSD). These neural crest-derived developmental anomalies are associated with altered expression of Hand2-target genes we have identified by gene profiling. A number of Hand2 direct target genes have been identified using ChIP and ChIP-on-chip analyses. We have identified and validated a number of genes related to cell migration, proliferation/cell cycle and intracellular signaling whose expression is affected by Hand2 deletion in the neural crest and which are associated with development of VSD and DORV. Our data suggest that Hand2 is a multifunctional DNA binding protein affecting expression of target genes associated with a number of functional interactions in neural crest-derived cells required for proper patterning of the outflow tract, generation of the appropriate number of neural crest-derived cells for elongation of the conotruncus and cardiac cushion organization. Our genetic model has made it possible to investigate the molecular genetics of neural crest contributions to outflow tract morphogenesis and cell differentiation.
Hand2(dHand); neural crest; bHLH; heart; outflow tract; cardiac cushion; cell type-specific gene regulation; gene microarray
The current study aimed to develop a reliable targeted array comparative genomic hybridization (aCGH) to detect microdeletions and microduplications in congenital conotruncal defects (CTDs), especially on 22q11.2 region, and for some other chromosomal aberrations, such as 5p15-5p, 7q11.23 and 4p16.3.
Twenty-seven patients with CTDs, including 12 pulmonary atresia (PA), 10 double-outlet right ventricle (DORV), 3 transposition of great arteries (TGA), 1 tetralogy of Fallot (TOF) and one ventricular septal defect (VSD), were enrolled in this study and screened for pathogenic copy number variations (CNVs), using Agilent 8 x 15K targeted aCGH. Real-time quantitative polymerase chain reaction (qPCR) was performed to test the molecular results of targeted aCGH.
Four of 27 patients (14.8%) had 22q11.2 CNVs, 1 microdeletion and 3 microduplications. qPCR test confirmed the microdeletion and microduplication detected by the targeted aCGH.
Chromosomal abnormalities were a well-known cause of multiple congenital anomalies (MCA). This aCGH using arrays with high-density coverage in the targeted regions can detect genomic imbalances including 22q11.2 and other 10 kinds CNVs effectively and quickly. This approach has the potential to be applied to detect aneuploidy and common microdeletion/microduplication syndromes on a single microarray.
SMAD4 acts as the converging point for TGFβ and BMP signaling in heart development. Here, we investigated the role of SMAD4 in heart development using a novel α skeletal muscle actin Cre recombinase (MuCre) transgenic mouse strain. Lineage tracing using MuCre/ROSA26LacZ reporter mice indicated strong Cre-recombinase expression in developing and adult heart and skeletal muscles. In heart development, significant MuCre expression was noted at E11.5 in the atrial, ventricular, outflow tract and atrioventricular canal myocardium, but not in the endocardial cushions. MuCre-driven conditional deletion of Smad4 in mice caused double outlet right ventricle (DORV), ventricular septal defect (VSD), impaired trabeculation and thinning of ventricular myocardium, and mid-gestational embryonic lethality. In conclusion, MuCre mice effectively delete genes in both heart and skeletal muscles, thus enabling the discovery that myocardial Smad4 deletion causes misalignment of the outflow tract and DORV.
heart; myogenesis; transforming growth factor beta; SMAD; Marfan syndrome.
To investigate an association between surface electrocardiographic (ECG) parameters and sustained ventricular tachycardia (VT) in children after repair of congenital heart disease (CHD), data were obtained and analyzed in three groups (group I, 7 postoperative patients with episode of sustained VT (4 tetralogy of Fallot (TOF), 2 double outlet right ventricle (DORV), 1 truncus arteriosus); group II, 14 children with postoperative TOF not associated with VT; group III, 14 normal children). Mean age at the onset of sustained VT was 129+/-77 months (range 60-232); mean age at corrective surgery, 44+/-33 months (range 10-102); mean follow-up period after surgery, 84+/-74 months (range 20-185); the duration from repair to the onset of sustained VT, range 1-185 months. Compared to group II and III, group I showed longer QRS duration (group I, 137+/-10 msec; group II, 114+/-22 msec; group III, 65+/-12 msec) and shorter corrected J to Tmax interval (group I, 209+/-24 msec; group II, 272+/-44 msec; group III, 249+/-18 msec). QT and corrected QT, J to Tmax interval, and their dispersions in group I and II are significantly different from those of group III. In conclusion, QRS duration and corrected J to Tmax interval could be helpful to predict ventricular tachycardia in postoperative CHD.
The aim of this investigation is to demonstrate that in Ebstein's Anomaly (EA) the right ventricle (RV) is affected in its three portions and to establish an anatomoechocardiographic correlation between the anatomic features and the equivalent echocardiographic images.
Thirty hearts with EA were studied. The alterations of each portions of the RV were described. Fifty adult patients with this anomaly were studied by echocardiography.
Anatomy: All hearts had atrial situs solitus, 27 had concordant atrioventricular connection and 3 discordant, of these 2 had transposition of the great arteries (TGA) and one double outlet right ventricle (DORV). The degree of tricuspid valve (TV) displacement showed a spectrum from I to III. The inlet of the RV was markedly thin in 27. The trabecular portion had multiples muscular bands in all. The outlet portion was dilated in 20 and stenotic in 5. In 25 atrial septal defects were found. Echocardiography: All patients had atrial situs solitus, 42 with concordant atrioventricular connection and 8 with discordant, of these last patients 5 had TGA and 3 DORV. The degree of TV displacement varied from I to III. The inlet of RV was markedly thin in 42. The trabecular portion had muscular bands in 45. The outlet portion was dilated in 31 and stenotic in 11. In 30 atrial septal defects were found.
The EA affects the whole RV and the anatomoechocardiographic correlation provides an appropriate understanding of echocardiographic images in terms of a precise diagnosis, therapeutic decisions and prognosis.
Biventricular repair is usually difficult to achieve in patients who have right atrial isomerism, which is typically associated with other complex cardiac anomalies. The procedure can be used in patients who have balanced ventricular structures. Herein, we report a successful surgical reconstruction, including biventricular repair, in a 4-year-old boy. The child's right atrial isomerism was associated with double-outlet right ventricle, a large atrial septal defect, a subaortic ventricular septal defect, valvular and infundibular pulmonary stenosis, left persistent superior vena cava, and hemiazygos continuation of an interrupted inferior vena cava. Balanced ventricles enabled biventricular repair, which we consider to be preferable to the Fontan procedure in such circumstances.
Abnormalities, multiple/surgery; cardiac surgical procedures/methods; child, preschool; coronary vessels/anatomy & histology; Fontan procedure; heart atria/abnormalities; heart defects, congenital/physiopathology/surgery; heart ventricles/abnormalities/physiopathology
Most of the patients with congenital heart diseases express the atrial myosin light chain 1 (ALC-1) in the right ventricle. We investigated the functional consequences of ALC-1 expression on the myosin cycling kinetics in the intact sarcomeric structure using multicellular demembranated fibers ("skinned fibers") from the right ventricular infundibulum of patients with Tetralogy of Fallot (TOF), double outlet right ventricle (DORV), and infundibular pulmonary stenosis (IPS), Force-velocity relation was analyzed by the constant-load technique at maximal Ca2+ activation (pCa 4.5). Half-time of tension development (t1/2) was investigated by monitoring contraction initiation upon photolytic release of ATP from caged-ATP in rigor. The patients investigated here expressed between 0 and 27% ALC-1. There was a statistically significant correlation between ALC-l and maximal shortening velocity (Vmax) which rose 1.87-fold from 1.2 muscle length per second (ML/s) to 2.25 ML/s in a normal (0% ALC-1) and diseased (19.9% ALC-1) ventricle. Half-time of tension development decreased 1.85-fold with increasing ALC-1 expression (t1/2) was 0.252 s and 0.136 s at 2 and 18.4% ALC-1, respectively). We conclude that the expression of ALC-1 in the human heart modulates cross-bridge cycling kinetics accelerating shortening velocity and isometric tension production.
The existence of a cavity of noninfectious origin between the annulus of the ascending aorta and the left ventricle is described. Aortic-left ventricular discontinuity occurred as a late complication of aortic valve replacement in an octogenarian. A patient with severe symptomatic aortic valve stenosis underwent aortic valve replacement and aortic root enlargement into the noncoronary sinus with pericardial tissue. Four months after her valve surgery the patient presented with shortness of breath. Angiocardiography and transoesophageal echocardiography revealed a complex, irregular pouch at the right coronary cusp side of the valve with paravalvular leakage back into the left ventricular outflow tract. Reoperation was performed, the disconnection between the aortic annulus and the left ventricle was repaired and the valve was replaced. The aortic-left ventricular discontinuity did not involve the area where aortic root enlargement was performed. No evidence of infection was found and the most likely cause of the reported complication is mechanical in nature.
aortic-left ventricular discontinuity; aortic valve replacement; complications; noninfectious
We aimed to determine outcomes for the Ross procedure in paediatric and adult patients, with particular emphasis on survival, complication and reintervention rates. A retrospective review of 101 patients who had the Ross procedure in a congenital cardiac surgical centre serving a population of approximately 2.5 million was performed. There were 69 adults and 32 children with a mean age of 24.8 ± 13.9 years. Indications for surgery were aortic stenosis (48), regurgitation (10), mixed disease (35) and complex left outflow tract obstruction (8). The mean follow-up duration was 4.7 ± 3.7 years. The mini-inclusion technique was used to incorporate the autograft, and in all cases, pulmonary homografts were placed in the right ventricular outflow tract. Sub-aortic resection was also performed in six and Ross–Konno operations in eight patients. There were no early deaths and there was one late death secondary to endocarditis. Freedom from reintervention was 92% at 5 years and 77% at 10 years. Children were significantly more likely to require reintervention (16%, 5 of 32 versus 4%, 3 of 69, P = 0.05). The Ross procedure carries low early and mid-term mortality, and reintervention rates appear acceptable. The Ross procedure should be considered a feasible alternative to prosthetic valves in patients who require aortic valve replacement.
Aortic valve; Mortality; Reoperation
To limit the trauma to the chest and to achieve a pleasing cosmetic result, we used 2 types of right anterolateral thoracotomy in 48 patients who required open-heart surgery: 1 was a curved incision along the lower edge of the right breast in women with developed breasts; the other was a slanted incision for men and children. These surgical procedures took place between July 1996 and November 1997. Intraoperatively, a right atriotomy was used to repair 11 atrial septal defects and 11 ventricular septal defects, 2 combined atrial and ventricular septal defects, 1 case of a single atrium, and 1 partial atrioventricular canal. A right ventricular outflow tract incision was used to repair 7 ventricular septal defects and 7 ruptured aortic sinus aneurysm. A combination of a right atriotomy and right ventricular outflow tract incision was used for 2 repairs of combined atrial and ventricular septal defects, 3 radical corrections of tetralogy of Fallot, and 2 radical corrections of trilogy of Fallot. A combined right and interatrial septal incision was used for 6 mitral valve replacements and 1 mitral valvuloplasty. Smooth bypass cannulation and satisfactory intracardiac exposure were achieved with the right anterolateral thoracotomy. There was no complication or mortality directly related to the incision. We believe that the right anterolateral thoracotomy is safer and more effective than the median sternotomy for many common congenital and acquired heart diseases. The thoracotomy causes less trauma and results in a cosmetic appearance that is more acceptable to the patient.
Out of 1610 children's hearts with congenital malformations there were 27 specimens showing double outlet right ventricle. Cases with dextrocardia, situs inversus, or l-venticular loop were excluded. Anatomical examination was performed with particular reference to the infundibular region, the great vessels, and the ventricular septum. The commonest associated malformations were ventricular septal defect and pulmonary stenosis. Aortic stenosis was the predominant finding in those cases dying in the neonatal period. An aortic conus was associated with pulmonary stenosis, ventricular septal defect, and d-transposition, a pulmonary conus with ventricular septal defect and a double conus with stenosis of either great vessel. The anterior vessel always had a muscular conus and the posterior vessel was commonly stenotic.
OBJECTIVE--The aim was to define the long term prognosis of isolated ventricular septal defect (VSD) with anteriorly malaligned outlet septum. DESIGN--Cohort study. SETTING--University hospital, tertiary medical care centre. PATIENTS--Between July 1986 and June 1993, 63 patients were studied with an isolated VSD and anteriorly malaligned outlet septum (59 perimembranous; 4 muscular outlet). MAIN OUTCOME MEASURES--The diagnosis of septal malalignment, aneurysmal transformation, right ventricular obstruction, subaortic ridge, and aortic valve prolapse was based on echocardiographic criteria, then confirmed by angiography in 33 patients and by surgery in 28. An actuarial curve for each event was obtained by Kaplan-Meier non-parametric analysis and the significance was examined by log-rank test. RESULTS--Aneurysmal transformation decreased the size of the VSD in 52% of the patients, but was also associated with the appearance of subaortic ridge (p < 0.05). Progressive obstruction in the right ventricle was observed in 51%, more often in those without aneurysmal transformation (p < 0.05). Aortic valve prolapse was quite common whether or not aneurysmal transformation occurred (33% and 23%, respectively). This was attributed to the location of the VSD and the anterior malalignment of the outlet septum. Surgery was performed in 28 patients at a median age of 50 months because of significant left to right shunt (n = 5), or the development of obstruction in right ventricle (n = 9), aortic valve prolapse (n = 3), or combinations (n = 11). The presence of subaortic ridge per se was not considered to be a surgical indication. CONCLUSIONS--Anteriorly malaligned VSDs have variable presentation. Careful echocardiographic evaluation is needed to identify various combinations of progressive right ventricular obstruction, aneurysmal transformation, subaortic ridge, or aortic valve prolapse. In extreme cases a patient may have a pathology complex comprising right ventricular outflow obstruction, subaortic ridge, aortic valve prolapse, and anteriorly malaligned VSD.
Twenty-two patients with transposition of the great arteries with or without ventricular septal defect and one with double outlet right ventricle, d-malposition, and severe pulmonary vascular obstructive disease were treated surgically. All were cyanosed and had very limited exercise tolerance. Preoperatively, systemic arterial oxygen saturation (SaO2) varied from 45 to 79% (mean 65), haemoglobin was 13 to 23 g/dl (mean 19). Pulmonary arteriolar resistance was 6.4 to 35 units m2 (mean 17). In the patients with a ventricular septal defect the Mustard operation was done without closure of the ventricular septal defect, and in the 3 patients with intact ventricular septum the Mustard operation was combined with creation of a ventricular septal defect. All patients survived the operation and improved. Postoperative SaO2 ranged from 75 to 96% (mean 89) and haemoglobin from 10.6 to 17.8 g/dl (mean 14.0). This improvement was significant (P less than 0.05). Five patients have had a postoperative cardiac catheterisation. The pulmonary arteriolar resistance remains high in all. Postoperative follow-up varies from 4 to 40 months (mean 14 months). So far there have been no late deaths and all patients remain improved.
The roles of posterior bulging of the interventricular septum (septal bulge) and of systolic septal mitral apposition in patients with simple transposition of the great arteries are not known. Cross sectional echocardiograms of 40 such patients were reviewed (after exclusion of those with fixed left ventricular outflow tract obstruction) and haemodynamic findings were compared with long and short axis measurements within the left ventricle. There was no significant correlation between the degree of septal bulge and systolic gradient across the left ventricular outflow tract, but septal bulge correlated weakly with systolic right ventricular pressure and inversely with pulmonary arteriolar resistance index. Systolic left ventricular outflow gradient was inversely related to the minimum systolic distance between the anterior mitral leaflet and interventricular septum. No patients without complete systolic apposition of the anterior mitral leaflet and interventricular septum had a left ventricular outflow gradient greater than 20 mm Hg. Conversely, even when cross sectional echocardiography showed apparently total obstruction of the left ventricular outflow tract at some time in systole there was often no significant gradient detected during haemodynamic study. In the short axis cuts closeness of the papillary muscles to the interventricular septum or to each other was unrelated to systolic gradient. This study shows that (a) cross sectional echocardiography can identify fixed obstruction of the left ventricular outflow tract in simple transposition of the great arteries; (b) the degree of septal bulge, unless complicated by fibrous thickening of the anterior mitral leaflet and interventricular septum, is unrelated to the gradient across the left ventricular outflow tract; (c) the absence of systolic septal/mitral apposition excludes a significant gradient at that site across the left ventricular outflow tract; and (d) papillary muscle geometry is unrelated to dynamic gradients across the left ventricular outflow in this condition.
Ventricular septal defect (VSD) can be associated with various complications such as aortic regurgitation (AR). AR in VSD come from a deficiency or hypoplasia of the conal septum which leads to abnormal apposition in diastole and prolapse of the poorly supported noncoronary or right coronary cusp through the VSD into the right ventricle resembling subpulmonic stenosis and subsequently results in distortion of the aortic valve and progressive AR. AR often increases in severity with age and it indicates a worse prognosis. Therefore, appropriate timing of surgical repair in progressive AR in VSD might be important. Until now, many earlier experiences about surgical repair of AR complicating VSD were on adolescents or young adults. We reported a case of AR in 48-year-old male patient with right coronary cusp prolapse complicating the subarterial type of VSD which was properly assessed by echocardiography and was successfully treated with surgical repair. Right coronary cusp or noncoronary cusp prolapse should be suspected in AR complicating VSD through proper echocardiographic assessment and the surgical repair on VSD and distorted aortic valve should be considered in the old patient, as well as the young.
Ventricular septal defect; Aortic valve; Aortic regurgitation; Prolapse
Two cases are described of a most unusual variant of two-chambered right ventricle. In both the ventricular septal defect was between the distal chamber of the right ventricle and the left ventricle. However the extensive dividing 'septum' between proximal and distal parts of the right ventricle converted the latter, haemodynamically, into part of the left ventricle. In the first case the distal chamber supported the aorta in the left anterior position, the pulmonary artery arising from the proximal part of the right ventricle. In the second the pulmonary artery arose from the distal chamber and the aorta from the proximal chamber. Though in both the ventriculoarterial connection was double outlet right ventricle, functionally there was arterial concordance in case 1 and discordance in case 2. A further disconcerting feature was the resemblance of the distal right ventricular chamber to the rudimentary chamber of a univentricular heart of left ventricular type.
Progressive cyanosis after banding of the pulmonary artery in infancy occurred in a child with transposition of the great arteries and a ventricular septal defect, and a Blalock-Taussig shunt operation had to be performed. At the time of correction a segment of pulmonary artery between the left ventricle and the band was found to be completely occluded so that continuity between the left ventricle and the pulmonary artery could not be restored. A Rastelli type of operation was not feasible as the ventricular septal defect was sited low in the muscular septum. Therefore, in addition to Mustard's operation, a Dacron conduit was inserted from the left ventricle to the main pulmonary artery to relieve the obstruction. Postoperative cardiac catheterization with angiocardiography indicated a satisfactory haemodynamic result. The patient remains well 11 months after the operation. This operation, a left ventricle to pulmonary artery conduit, may be used as an alternative procedure in patients with transposition of the great arteries, intact interventricular septum, and obstruction to the left ventricular outflow, if the obstruction cannot be adequately relieved.