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1.  Hybrid Palliation for Neonates With Hypoplastic Left Heart Syndrome: Current Strategies and Outcomes 
Korean Circulation Journal  2010;40(3):103-111.
In the last decade the hybrid procedure has emerged as an alternative stage I palliation in neonates with hypoplastic left heart syndrome (HLHS). This review discusses the historical aspect, surgical and interventional techniques, current outcomes and future direction of this procedure. Hybrid palliation yields equivalent but not superior stage I palliation survival and comparable 1-year survival to conventional Norwood palliation, comparable prestage II hemodynamics and pulmonary artery growth, and preserved ventricular function in stage II palliation. Hybrid palliation utilizes significantly less resource and shortens postoperative recovery. In comprehensive stage II palliation the impact of pulmonary artery reconstruction on subsequent pulmonary artery growth has not been determined and should be further investigated. A prospective, randomized trial is warranted to compare these two surgical strategies for neonates with hypoplastic left heart syndrome.
PMCID: PMC2844974  PMID: 20339493
Hypoplastic left heart syndrome; Stents; Heart defects; Congenital
2.  Cardiovascular magnetic resonance of pulmonary artery growth and ventricular function after Norwood procedure with Sano modification 
For hypoplastic left heart syndrome (HLHS), there have been concerns regarding pulmonary artery growth and ventricular dysfunction after first stage surgery consisting of the Norwood procedure modified with a right ventricle-to-pulmonary artery conduit. We report our experience using cardiovascular magnetic resonance (CMR) to determine and follow pulmonary arterial growth and ventricular function in this cohort.
Following first stage palliation, serial CMR was performed at 1 and 10 weeks post-operatively, followed by cardiac catheterization at 4 – 6 months. Thirty-four of 47 consecutive patients with HLHS (or its variations) underwent first stage palliation. Serial CMR was performed in 20 patients. Between studies, ejection fraction decreased (58 ± 9% vs. 50 ± 5%, p < 0.05). Pulmonary artery growth occurred on the left (6 ± 1 mm vs. 4 ± 1 mm at baseline, p < 0.05) but not significantly in the right. This trend continued to cardiac catheterization 4–6 months post surgery, with the left pulmonary artery of greater size than the right (8.8 ± 2.2 mm vs. 6.7 +/- 1.9 mm, p < 0.05). By CMR, 5 had pulmonary artery stenoses initially, and at 2 months, 9 had stenoses. Three of the 9 underwent percutaneous intervention prior to the second stage procedure.
In this cohort, reasonable growth of pulmonary arteries occurred following first stage palliation with this modification, although that growth was preferential to the left. Serial studies demonstrate worsening of ventricular function for the cohort. CMR was instrumental for detecting pulmonary artery stenosis and right ventricular dysfunction.
PMCID: PMC2491614  PMID: 18601747
3.  Improved early ventricular performance with a right ventricle to pulmonary artery conduit in stage 1 palliation for hypoplastic left heart syndrome: evidence from strain Doppler echocardiography 
Heart  2004;90(2):191-194.
Objective: To quantify non-invasively right ventricular (RV) performance in infants after stage 1 palliation for hypoplastic left heart syndrome (HLHS).
Design: Prospective, observational study with two dimensional and strain Doppler echocardiography.
Setting: Single tertiary paediatric cardiology centre.
Patients: Convenience sample of nine consecutive infants with HLHS. Four whose surgery involved a systemic to pulmonary artery (S-PA) shunt were compared with five whose surgery incorporated a right ventricle to pulmonary artery (RV-PA) conduit.
Methods: Basal RV free wall longitudinal strain rate, systolic strain (ε), and RV percentage area change were calculated during a single assessment between 27–50 days after surgery.
Results: Cardiopulmonary bypass time was longer in patients who underwent RV-PA (226 (30) minutes v 181 (18) minutes, p  =  0.03), but cross clamp time, duration of ventilation, and inotrope use did not differ. Two patients in the S-PA group died, on days 29 and 60 after surgery. Peak systolic strain rate (−1.24 (0.19)/s v −0.91 (0.21)/s, p  =  0.048), peak ε (−17.8 (1.8)% v –13.4 (2.0)%, p  =  0.01), and RV percentage area change (56 (6)% v 25 (6)%, p < 0.01) were all greater among RV-PA patients. These indices also tended to be greater in survivors as a group. Ventricular loading conditions (oxygen saturations, diuretic treatment, and blood pressure) were similar in both groups.
Conclusion: Strain Doppler echocardiography shows improved RV longitudinal systolic contractility in patients during convalescence after the RV-PA modification of stage 1 palliation for HLHS compared with those with an S-PA shunt.
PMCID: PMC1768056  PMID: 14729795
strain Doppler echocardiography; hypoplastic left heart syndrome; ventricular function
4.  Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome. 
British Heart Journal  1993;69(6):551-555.
OBJECTIVE--To assess the feasibility of pulmonary artery banding, atrial septectomy or septostomy, and percutaneous stenting of the arterial duct in babies with the hypoplastic left heart syndrome. PATIENTS--Four infants with hypoplastic left heart syndrome. SETTING--Two supraregional paediatric cardiac centres. METHODS--Ductal patency was maintained initially with prostaglandin E. Banding of the proximal branch pulmonary arteries was performed through a median sternotomy and open atrial septectomy was performed if balloon septostomy was not. Stainless steel stents (Johnson & Johnson) mounted in a balloon catheter were implanted into the arterial duct under radiographic control and expanded to a diameter of approximately 8 mm, prostaglandin treatment was then stopped. RESULTS--All the patients survived the immediate postoperative period and maintenance of wide ductal patency was achieved in three of the four patients by stent implantation. Two weeks after the procedure two babies died of right ventricular failure and respiratory infection: some distal ductal constriction had occurred in one where the stent was not quite sufficiently distally placed. One child was discharged home 15 days after treatment and was well at follow up at age 16 weeks and one was stable but required diuretic therapy five weeks after the procedure. CONCLUSIONS--This new approach is technically feasible. At least in the short term it seems to offer hope of effective palliation for the hypoplastic left heart syndrome and it warrants further study.
PMCID: PMC1025171  PMID: 7688231
5.  Stenting of the ductus arteriosus and banding of the pulmonary arteries: basis for various surgical strategies in newborns with multiple left heart obstructive lesions 
Heart  2003;89(6):645-650.
Objective: To present an institutional experience with stent placement in the arterial duct combined with bilateral banding of the pulmonary artery branches as a basis for various surgical strategies in newborns with hypoplastic left heart obstructive lesions.
Design: Observational study.
Setting: Paediatric heart centre in a university hospital.
Patients: 20 newborns with various forms of left heart obstructive lesions and duct dependent systemic blood flow.
Interventions: Patients underwent percutaneous ductal stenting and surgical bilateral pulmonary artery banding. Atrial septotomy by balloon dilatation was performed as required, in one premature baby by the transhepatic approach.
Main outcome measures: Survival; numbers of and reasons for palliative and corrective cardiac surgery.
Results: One patient died immediately after percutaneous ductal stenting. One patient died in connection with the surgical approach of bilateral pulmonary banding. Stent and ductal patency were achieved for up to 331 days. Two patients underwent heart transplantation and two patients died on the waiting list. Ten patients had a palliative one stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5–6 months. There was one death. One patient is still awaiting this approach. Two patients received biventricular repair. In one, biventricular repair will soon be provided.
Conclusions: Stenting the arterial duct combined with bilateral pulmonary artery banding in newborns with hypoplastic left heart or multiple left heart obstructive lesions allows a broad variation of surgical strategies depending on morphological findings, postnatal clinical conditions, and potential ventricular growth.
PMCID: PMC1767699  PMID: 12748222
ductus arteriosus; stent; Norwood repair; hypoplastic left heart
6.  Congenital heart disease in the newborn requiring early intervention 
Korean Journal of Pediatrics  2011;54(5):183-191.
Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.
PMCID: PMC3145901  PMID: 21829408
Congenital heart disease; Early intervention
7.  Congenital absence of ductus arteriosus--an autopsy case. 
Isolated absence of the ductus arteriosus is extremely rare condition although the ductus arteriosus may be hypoplastic or aplastic in association with other aortic arch anomalies. Authors described a case of isolated agenesis of the ductus arteriosus documented by postmortem examination of a newborn infant who died of pneumonia following operation for a large omphalocele. The heart showed ventricular septal defect. However, no other cardiovascular anomalies were associated in this case. There were three vessels that were taking off from the aorta consisted of the right brachiocephalic artery, left common carotid-artery and left subclavian artery. The anteriorly located pulmonary artery was divided into the right and left pulmonary arteries. There was no connection of vessel between the pulmonary artery and the aorta.
PMCID: PMC3053659  PMID: 3267353
8.  Hypoplastic Left Heart Syndrome: Report of an Operation Utilizing the Ductus Arteriosis in Outflow Reconstruction 
Texas Heart Institute Journal  1986;13(4):463-467.
An operation utilizing the ductus arteriosus to construct an outflow tract from the right ventricle is described. This operation uses the right ventricle for pulsatile flow to both systemic and pulmonary arteries, provides complete reconstruction of the ascending aorta and arch, and delivers predictable pulmonary blood flow. Although unsuccessful in this case report, which involves a hypoplastic left heart syndrome patient, the technique may provide successful palliation with the use of ductal tissue. This method has not been previously described. (Texas Heart Institute Journal 1986; 13:463-467)
PMCID: PMC324679  PMID: 15227356
Hypoplastic left heart syndrome; ductus arteriosus
9.  Comparison of Shunt Types in the Norwood Procedure for Single-Ventricle Lesions 
The New England journal of medicine  2010;362(21):1980-1992.
The Norwood procedure with a modified Blalock–Taussig (MBT) shunt, the first palliative stage for single-ventricle lesions with systemic outflow obstruction, is associated with high mortality. The right ventricle–pulmonary artery (RVPA) shunt may improve coronary flow but requires a ventriculotomy. We compared the two shunts in infants with hypoplastic heart syndrome or related anomalies.
Infants undergoing the Norwood procedure were randomly assigned to the MBT shunt (275 infants) or the RVPA shunt (274 infants) at 15 North American centers. The primary outcome was death or cardiac transplantation 12 months after randomization. Secondary outcomes included unintended cardiovascular interventions and right ventricular size and function at 14 months and transplantation-free survival until the last subject reached 14 months of age.
Transplantation-free survival 12 months after randomization was higher with the RVPA shunt than with the MBT shunt (74% vs. 64%, P = 0.01). However, the RVPA shunt group had more unintended interventions (P = 0.003) and complications (P = 0.002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (±SD) follow-up period of 32±11 months showed a nonsignificant difference in transplantation-free survival between the two groups (P = 0.06). On nonproportional-hazards analysis, the size of the treatment effect differed before and after 12 months (P = 0.02).
In children undergoing the Norwood procedure, transplantation-free survival at 12 months was better with the RVPA shunt than with the MBT shunt. After 12 months, available data showed no significant difference in transplantation-free survival between the two groups. ( number, NCT00115934.)
PMCID: PMC2891109  PMID: 20505177
10.  Perioperative Management of Infants Undergoing Fundoplication and Gastrostomy After Stage I Palliation of Hypoplastic Left Heart Syndrome 
Pediatric cardiology  2012;33(5):697-704.
Infants with hypoplastic left heart syndrome (HLHS) represent a high-risk population when they present for noncardiac surgery. To assist clinicians in the care of these infants, we present our experience of 36 HLHS patients who underwent abdominal surgery after stage I palliation. We reviewed patients with HLHS who underwent gastrostomy and/or fundoplication after stage I palliation during an 18-month period. We assessed the impact of preoperative echocardiographic predictors and regional anesthesia on use of intraoperative inotropes, extubation in the OR, perioperative instability, postoperative escalation of care, and length of hospital and intensive care unit stay. Of 39 abdominal operations, all but 2 were performed with open laparotomy. There was a positive association between regional anesthesia and instability during induction. Escalation of respiratory care occurred in 9 (23.1%) cases, and escalation of hemodynamic care occurred in 6 (15.4%) cases. Neoaortic valve insufficiency was associated with increased length of stay, and ventricular outflow obstruction was associated with escalation of hemodynamic care. Extubation in the OR was successful in 31 cases (79.5%). In-hospital death occurred in 1 patient (2.7%). HLHS infants often undergo abdominal surgery, but intraoperative instability and need for escalation of care is common. Specific echocardiographic findings were associated with length of stay and escalation of care. Regional anesthesia was associated with transient intraoperative instability but not with other adverse outcomes.
PMCID: PMC3413202  PMID: 22314367
Hypoplastic left heart syndrome; Abdominal surgery; Fundoplication; Gastrostomy
11.  Extrinsic Compression of the Left Coronary Ostium by the Pulmonary Trunk 
Texas Heart Institute Journal  2010;37(1):95-98.
Extrinsic compression of the left main coronary artery by a massively dilated pulmonary artery in patients who have severe pulmonary hypertension can lead to significant myocardial ischemia. A 58-year-old man with a large patent ductus arteriosus and Eisenmenger syndrome presented with angina at rest and worsening heart failure of 3 months' duration. The new symptoms were recognized to be secondary to extrinsic compression of the left main coronary artery ostium by a dilated main pulmonary artery and were successfully relieved by the placement of a metallic stent in the affected segment of the left main coronary artery. Multislice computed tomographic imaging after 6 months showed stent patency and the intimate relation of the stented vessel to the dilated main pulmonary trunk. We discuss diagnostic and management issues pertaining to this uncommon clinical entity.
PMCID: PMC2829817  PMID: 20200637
Angina pectoris/etiology; angioplasty, transluminal, percutaneous coronary; constriction, patho-logic/etiology; coronary stenosis/etiology; dilatation, pathologic/complications; ductus arteriosus, patent; Eisenmenger complex/ complications; hypertension, pulmonary/complications; stents; tomography, X-ray computed
12.  Patent ductus arteriosus closure using an Amplatzer™ ventricular septal defect closure device 
The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs.
PMCID: PMC3716504  PMID: 24294051
Amplatzer™ ventricular septal defect closure device; Patent ductus arteriosus; Transcatheter closure
13.  Vasoreactive Response to Maternal Hyperoxygenation (MH) in the Fetus with Hypoplastic Left Heart Syndrome (HLHS) 
Cardio-pulmonary interactions play an important role in the pathophysiology of hypoplastic left heart syndrome (HLHS). Pulmonary vasculopathy has been identified, especially in those with restrictive/intact atrial septum (R/IAS). Responsiveness of the pulmonary vasculature to maternal hyperoxygenation (MH) may provide a tool to assess the degree of pulmonary vasculopathy present prior to birth.
Methods and Results
Doppler echocardiography was performed in 27 normal and 43 HLHS fetuses. In HLHS, sampling was repeated after 10 minutes of MH with 60% FiO2, and after 5 minutes of recovery. Sampling was performed in the proximal, mid-portion, and distal branch pulmonary artery (PA). Pulsatility index (PI) was used as a measure of vascular impedance. Of the HLHS fetuses, 34 had an open inter-atrial septum and 9 had a R/IAS. At birth, 5 fetuses underwent immediate intervention on the inter-atrial septum. Middle cerebral artery PI was lower in HLHS vs. normals (p<0.001). There was no difference in umbilical artery, ductus arteriosus, or branch PA PI between normals and HLHS. MH led to a significant decrease in PI at each of the PA sites sampled in fetuses with an open atrial septum (p<0.001); however, there no was significant change in the PI in fetuses that required immediate intervention on the atrial septum at birth. Using a cutoff value of <10% vasoreactivity, the sensitivity of MH testing for determining need for immediate intervention at birth is 100% [0.46-1.0], specificity 94% [0.78-.99], positive predictive value 71% [0.30-0.95], and negative predictive value 100% [0.86-1.0]. No untoward effects were seen with MH.
PA vasoreactivity to MH occurs in the fetus with HLHS. MH testing accurately identifies fetuses requiring urgent postnatal intervention at birth and may be used to select candidates for fetal atrial septoplasty.
PMCID: PMC3070268  PMID: 20044513
congenital heart defects; fetal echocardiography; oxygen; hypoplastic left heart syndrome
14.  Transcatheter Treatment of “Pulmonary Artery Hypertension” due to Patent Ductus Arteriosus and Pulmonary Artery Stenosis 
Texas Heart Institute Journal  2006;33(3):383-385.
The association between large, left-sided patent ductus arteriosus and severe, peripheral, right pulmonary artery stenosis with no other cardiac malformation is an unreported condition that might be misdiagnosed as pulmonary hypertension due to long-standing ductal shunt. A 57-year-old man with supposed hypertensive patent ductus arteriosus underwent confirmatory cardiac catheterization. At angiography, a severe pre-hilar right pulmonary artery stenosis (peak pressure gradient, 65 mmHg) was found to complicate the hemodynamic picture of a moderate-to-large patent ductus arteriosus (QP/QS, 1.7:1), by causing pulmonary hypertension (mean pressure, 65 mmHg) and left-to-right pulmonary flow imbalance. Both lesions were treated in a single procedure of right pulmonary artery stenting and patent ductus arteriosus closure, after which the pulmonary artery pressure significantly decreased (mean, 35 mmHg). In our opinion, a thorough hemodynamic evaluation followed by pulmonary angiography should be mandatory before proceeding to patent ductus arteriosus closure in the adult patient who has “hypertensive” ductus, in whom possible associated malformations can be missed due to a poor echocardiographic window.
PMCID: PMC1592282  PMID: 17041703
Blood vessel prosthesis implantation; constriction, pathologic; diagnostic errors; ductus arteriosus, patent/complications/therapy; echocardiography, Doppler; embolization, therapeutic; heart catheterization; pulmonary artery/abnormalities; stents
15.  Surgical treatment of pulmonary atresia with intact ventricular septum. 
British Heart Journal  1976;38(9):957-960.
Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. All died, 19 early and 4 late. Of 13 patients who received a systemic-pulmonary artery shunt, 4 combined with surgical atrial septectomy, there are only 2 long-term survivors both of whom were children who had had a Waterston anastomosis. Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. If the Potts anastomosis appears satisfactory the persistent ductus arteriosus is ligated. This scheme was used in 23 infants, with 4 early deaths and 2 late deaths. Of 17 survivors, further shunts were required in 4 children. One child has had a formal repair, with insertion of valves in both tricuspid and pulmonary areas. We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.
PMCID: PMC483111  PMID: 971378
16.  Fetal Cerebrovascular Resistance and Neonatal EEG Predict 18-month Neurodevelopmental Outcome in Infants with Congenital Heart Disease 
The purpose of this study was to investigate early markers of risk for neurobehavioral compromise in congenital heart disease (CHD) survivors.
Fetuses < 24 wks gestational age (GA) were enrolled in this prospective pilot study for serial Doppler assessment of the middle cerebral and umbilical artery. The cerebral-to-placental resistance ratio (CPR) and MCA pulsatility index (PI) z-scores for GA were calculated. After birth, subjects underwent high-density (128-lead) electroencephalogram (EEG) and beta frequency (12–24Hz) band EEG power, a measure of local neural synchrony, was analyzed. Neurodevelopment was assessed at 18-months with the Bayley Scales of Infant Development III (BSID).
13 subjects were enrolled: 4 with hypoplastic left heart syndrome (HLHS), 4 with transposition of the great arteries (TGA), and 5 with tetralogy of Fallot (TOF). Compared with subjects with normal CPR, those with CPR<1(N=7) had lower mean BSID cognitive scores (91.4±4.8 vs. 99.2±3.8, p=.008). Fetal MCA PI z-score also correlated with BSID cognitive score (r=.589, p=0.044) as did neonatal EEG left frontal polar (r=.58, p=.037) and left frontal (r=.77,p=.002) beta power. Furthermore, fetal Doppler measures were associated with EEG power: fetuses with CPR<1 had lower left frontal polar (t=2.36, p=.038) and left frontal (t=2.85, p=.016) beta power as newborns compared with fetuses with normal CPR, and fetal MCA PI z-score correlated with neonatal EEG left frontal polar (r=.596, p=.04) and left frontal (r=.598, p=.04) beta power.
In CHD fetuses with HLHS, TGA, and TOF, abnormal cerebrovascular resistance predicted decreased neonatal EEG left frontal beta power and lower 18-mo cognitive development scores.
PMCID: PMC3612978  PMID: 22351034
congenital heart disease; neurodevelopment; fetal cerebral Doppler; fetal cerebrovascular resistance; hypoplastic left heart syndrome; tetralogy of Fallot; transposition of the great arteries; neonatal high-density electroencephalogram
17.  Coronary artery grafting in infants 
Coronary artery bypass grafting (CABG) with cardiac vale repair is an uncommon surgery in infants. CABG is technically demanding in infants due to the small size not only of the coronary arteries but also the potential graft arteries. The short and long-term outcome of surgery is not known and thus has largely been avoided.
We report the case histories of two infants in whom CABG was undertaken successfully as a life-saving measure. Case 1: This infant needed an arterial switch operation after which the right coronary artery (RCA) was stenosed resulting in low cardiac output. After the right internal mammary artery (RIMA) was used to anastamose the RCA, the hemodynamic status improved drastically. Case 2: This infant underwent surgical correction for Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA). Postoperatively, she was in low cardiac output. She was found to have an occluded left coronary artery and mitral regurgitation (MR). After she underwent left internal mammary artery (LIMA) to Left Anterior Descending (LAD) anastamosis and mitral valve repair, the clinical condition improved dramatically.
CABG is an uncommon operation in infants. This surgery is technically difficult. The long term results are not known and there are very few reports for the same. Though such an operation is best avoided, it can be used as a desperate life saving measure.
PMCID: PMC3232603  PMID: 22368554
18.  Fate of infants with transposition of the great arteries in relation to balloon atrial septostomy. 
British Heart Journal  1984;51(4):371-376.
The palliation afforded by balloon atrial septostomy to 124 infants with transposition of the great arteries was assessed in terms of survival to 6 months of age without the need for further intervention. Prediction of success or failure in relation to palliation was significantly affected by the presence of associated ventricular septal defect, left ventricular outflow tract obstruction, or persistent ductus arteriosus and by the maximum volume of balloon used to perform the septostomy. There was a significant association between balloon volume and size of atrial defect found at subsequent surgery or necropsy.
PMCID: PMC481516  PMID: 6704258
19.  Coil occlusion of systemic venous collaterals in hypoplastic left heart syndrome 
Heart  2002;88(2):167-169.
Objective: To assess the frequency of systemic venous collaterals to the atria, which may cause desaturation, after stage II reconstructive surgery for hypoplastic left heart syndrome (HLHS) and to determine whether coil occlusion prevents the need for surgical ligation.
Design: Prospective interventional study.
Setting: Tertiary referral centre.
Patients: 27 children with HLHS undergoing cardiac catheterisation between October 1996 and February 2001.
Interventions: 19 children were catheterised prestage II, 1 poststage II, and 17 prestage III. Aortic oxygen saturation (SaAo) and pulmonary artery pressure (pPA) were recorded. Angiography was performed into the left internal jugular vein to look for venous collaterals. If present, they were occluded with Cook MReye coils. Angiography was repeated to confirm occlusion, and SaAo and pPA were remeasured.
Results: Collaterals were found in 7 of 27 children: 1 poststage II and 6 prestage III. These were occluded with 1–3 coils without complication. Mean (SE) SaAo before occlusion was 80.2 (2.1)% in those with collaterals compared with 88.7 (1.0)% in those without (p = 0.007). There was no difference in mean pPA between the two groups. After coil occlusion mean SaAo rose to 83.8 (1.8)% (p = 0.007) and mean pPA rose from 12.5 (1.5) to 14.5 (1.8) mm Hg (p = 0.02). None required surgical ligation.
Conclusion: Angiography should be performed at catheterisation before stage II and III surgery for HLHS to exclude systemic venous collaterals. If present, they may be safely and effectively occluded with coils to improve saturation and prevent the need for subsequent surgical ligation.
PMCID: PMC1767215  PMID: 12117847
hypoplastic left heart syndrome; venous collaterals; cardiac catheterisation; coil occlusion
20.  Moderate altitude is not associated with adverse postoperative outcomes for patients undergoing bidirectional cavopulmonary anastomosis and Fontan operation: A comparative study among Denver, Edmonton, and Toronto 
Outcomes of patients with single ventricle physiology undergoing cavopulmonary palliations depend on pulmonary vascular resistance (PVR) and have been suggested to be adversely affected by living at elevated altitude. We compared the pulmonary hemodynamic data in correlation with postoperative outcomes at the 3 centers of Denver, Edmonton, and Toronto at altitudes of 1604, 668, and 103 meters, respectively.
Hemodynamic data at pre-bidirectional cavopulmonary anastomosis (BCPA) and pre-Fontan catheterization between 1995 and 2007 were collected. Death from cardiac failure or heart transplantation in the same period was used to define palliation failure.
There was no significant correlation between altitude (ranged from 1 to 2572 meters) and PVR, pulmonary artery pressure (PAP) or transpulmonary gradient (TPG) at pre-BCPA and pre-Fontan catheterization. BCPA failure occurred in 11 (9.2%) patients in Denver, 3 (2.9%) in Edmonton, and 34 (11.9%) in Toronto. Fontan failure occurred in 3 (6.1%) patients in Denver, 5 (7.2%) in Edmonton, and 11 (7.0%) in Toronto. There was no significant difference in BCPA and Fontan failure among the 3 centers. BCPA failure positively correlated with PVR and the presence of a right ventricle as the systemic ventricle. Fontan failure positively correlated with PAP and TPG.
Moderate altitude is not associated with an increased PVR or adverse outcomes in patients with a functional single ventricle undergoing BCPA and the Fontan operation. The risk factors for palliation failure are higher PVR, PAP, and TPG and a systemic right ventricle, but not altitude. Our study reemphasizes the importance of cardiac catheterization assessments of pulmonary hemodynamics before BCPA and Fontan operations.
PMCID: PMC3800264  PMID: 23353110
21.  Multiple Ventricular Septal Defects: A New Strategy 
Introduction: A multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs).
Materials and Methods: From 2004 to 2012 17 consecutive children (3 premature, 14 infants), mean age 3.2 months (9 days–9 months), mean body weight 4.2 kg (3.1–6.1 kg), with multiple VSDs underwent Pulmonary Artery Banding (PAB) with an adjustable FloWatch-PAB®. Associated cardiac anomalies included patent ductus arteriosus (1), aortic coarctation (2), hypoplastic aortic arch (2), and left isomerism (3). Five patients (5/17 = 29.4%) required pre-operative mechanical ventilation, with a mean duration of 64 days (7–240 days)
Results: There were no early or late deaths during a mean follow-up of 48 months (7–98 months), with either FloWatch removal or last observation as end-points. FloWatch-PAB® adjustments were required in all patients: a mean of 4.8 times/patient (2–9) to tighten the PAB, and a mean of 1.1 times/patient (0–3) to release the PAB with the patient’s growth. After a mean interval of 29 months (8–69 months) 10/17 (59%) patients underwent re-operation: 7/10 PAB removal, with closure of a remaining unrestrictive VSD in 6 (peri-membranous in 3 patients, mid-muscular in 2, and inlet in 1) and Damus–Kaye–Stansel, bi-directional Glenn, and atrial septectomy in 1; 3/9 patients required only PAB removal. All muscular multiple VSDs had closed in all 10 patients. PA reconstruction was required in 1/10 patient. In 5/7 of the remaining patients with the PAB still in situ, all muscular VSDs had already closed. The only 2 patients with persistent muscular multiple VSDs are the 2 patients with the shortest follow-up.
Conclusion: This reproducible new strategy with an adjustable PAB simplifies the management of infants with multiple VSDs and provides the following advantages: (a) good results (0% mortality), delayed surgery with a high incidence (15/17 = 88%) of spontaneous closure of multiple muscular VSDs, and facilitated closure of residual unrestrictive VSD (peri-membranous, mid-muscular, or inlet) at an older age and higher body weight; PAB with FloWatch-PAB® and its subsequent removal can potentially be the only procedure required for Swiss cheese multiple VSDs without an associated peri-membranous unrestrictive VSD.
PMCID: PMC3860893  PMID: 24400262
congenital heart defects; multiple ventricular septal defects; palliation; pulmonary artery banding; surgical repair
22.  Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution. 
British Heart Journal  1976;38(8):821-826.
Aortic valve atresia is usually associated with severe hypoplasia of the left ventricle and mitral apparatus. Palliative procedures, including atrial septectomy, pulmonary artery-aorta anastomosis, and pulmonary artery banding, have been performed in some patients. However, because of the distinct underdevelopment of the left heart, the long-term prognosis must be guarded even in the survivors of these procedures. Rarely, aortic valve atresia is found with adequate left heart chambers. Because very few of these patients are described, the clinical, angiocardiographic, and the pathological findings in two patients are presented, with comments on palliation in the neonatal period, and the potential for ventriculoaortic reconstitution.
PMCID: PMC483094  PMID: 61758
23.  Predictors of Retrograde Aortic Arch Obstruction After Hybrid Palliation of Hypoplastic Left Heart Syndrome 
Pediatric cardiology  2010;32(1):10.1007/s00246-010-9820-8.
A potential complication after hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) is retrograde aortic arch obstruction (RAAO). This can lead to increased morbidity and unplanned surgical or interventional procedures in the interstage period. This study aimed to identify potential predictors of RAAO by analyzing initial echocardiograms and angiograms before hybrid stage 1 palliation. For this study, 96 patients who underwent hybrid stage 1 palliation between July 2002 and July 2009 were reviewed, 68 of which had standard HLHS and met the inclusion criteria. The initial echocardiogram, hybrid stage 1 angiograms, and follow-up echocardiograms were reviewed. Anatomic and hemodynamic measurements were obtained by both modalities, and comparisons were made between those who developed RAAO and those who did not. Of the 68 patients, 20 (29%) had RAAO. The mean aortic root size was smaller for the patients who had RAAO (3.6 vs 4.4 mm; p = 0.036). The angiographic angle between the aortic isthmus and the patent ductus arteriosus (PDA) was significantly larger in the RAAO group (86° vs 63°; p = 0.008). The retrograde aortic arch velocities were higher in the RAAO group. Patients with RAAO have a smaller aortic root and higher retrograde velocities on initial echocardiogram. Patients with RAAO show a larger angle between the retrograde arch and PDA on angiogram. Because RAAO is an important potential complication after hybrid stage 1 palliation for HLHS, identification of predictors of RAAO may lead to improved care and outcome for patients with RAAO.
PMCID: PMC3818711  PMID: 20957474
Hybrid stage 1 palliation; Hypoplastic left heart syndrome; Retrograde aortic arch obstruction
24.  Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy? 
To determine whether a cardiac diagnosis is a predictor of neurodevelopmental outcomes after infant cardiac surgery.
Infants with ventricular septal defect (VSD), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), and hypoplastic left heart syndrome (HLHS) in a study of apolipoprotein E (APOE) polymorphisms, and neurodevelopmental outcome underwent neurodevelopmental and genetic evaluation at 4 years of age. The domains tested included cognition, language, speech, memory, executive function, visual-motor, fine motor, and reading and math skills.
Testing was completed in 178 patients with normal genetic evaluations: VSD (n = 26), TOF (n = 44), TGA (n = 41), and HLHS (n = 67). No differences were found in gestational age, ethnicity, APOE genotype, socioeconomic status, or maternal education among groups. Patient age at the first surgery was significantly lower for patients with TGA and HLHS compared with those with TOF and VSD. The postoperative length of stay was significantly longer for HLHS than all other groups and for TGA compared with TOF and VSD. HLHS correlated significantly with the use of deep hypothermic circulatory arrest and multiple operations. The mean scores for each domain were within normal limits for all groups. Compared with the other patients, those with HLHS had significantly lower scores for cognition, fine motor skills, executive function, and math skills. No significant differences were found among the TGA, TOF or VSD patients for any domain. Significant impairments in at least 1 domain were identified in 8% (2/25) of patients with VSD, 20% (8/41) with TOF, 17% (7/41) with TGA, and 18% (12/65) with HLHS. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain.
The mean scores for the neurodevelopmental outcomes domains tested were in the normal range for preschool children with no recognized genetic syndromes after surgery for VSD, TOF, TGA, and HLHS. In each diagnostic group, the number of children with impairments in at least 1 domain increased compared with the general population. Unadjusted neurodevelopmental outcomes for HLHS were lower for cognition, fine motor skills, executive function, and math skills compared with the other patients. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain. The specific cardiac diagnosis determines a large portion of the variation in these covariates. Therefore, although HLHS did predict for poorer outcomes in some domains, it did not add predictive power to the other factors considered.
PMCID: PMC3278908  PMID: 20951391
25.  Pan-Nitinol Occluder and Special Delivery Device for Closure of Patent Ductus Arteriosus 
Texas Heart Institute Journal  2013;40(1):30-33.
The aim of this study was to evaluate a new type of occluder for patent ductus arteriosus.
Patent ductus arteriosus was established in a canine model by anastomosing a length of autologous jugular vein to the descending aorta and the left pulmonary artery in an end-to-side fashion. Transcatheter closure of each patent ductus arteriosus was performed on 10 dogs, which were then monitored for as long as 6 months with aortography, echocardiography, and histologic evaluation.
Transcatheter closure with use of the novel pan-nitinol device was successful in all canine models. Postoperative echocardiography showed that the location and shape of the occluders were normal, without any residual shunting. Further histologic evaluation confirmed that the occluder surface was completely endothelialized 3 months after implantation.
Transcatheter patent ductus arteriosus closure with the pan-nitinol occluder can be performed safely and successfully in a canine model and shows good biological compatibility and low mortality rates.
PMCID: PMC3568284  PMID: 23466429
Alloys/administration & dosage; biocompatible materials; disease models, animal; dogs; ductus arteriosus, patent/therapy; heart catheterization/instrumentation/methods; heart septal defects, ventricular/therapy; nitinol/chemistry; prosthesis design; prosthesis implantation/instrumentation/methods; septal occluder device; stainless steel/chemistry

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