Anatomy of functionally single ventricle.

Hearts that have previously been called univentricular hearts, or single ventricles, can be described as having a univentricular atrioventricular connection. Most such hearts have two ventricular chambers, albeit one is small and incomplete-lacking an inlet component. The atriums of these hearts connect only to one of these ventricular chambers, which is usually the larger and dominant ventricle. Other hearts, with biventricular atrioventricular connections, may have hypoplasia of one ventricle, making it impossible to restore a biventricular circulation and such hearts are functionally univentricular. The term "functionally single ventricle" (or functionally univentricular heart) encompasses both these categories of malformation.

Anatomy of discordant atrioventricular connections.

The term discordant atrioventricular connections refers to the situation in which the ventricles are connected inappropriately to the atrial chambers. In most instances, the connections of the great arteries are also abnormal, with the aorta and the pulmonary trunk arising from morphologically inappropriate ventricles. This combination results in the presence of so-called congenitally corrected transposition. Double-outlet right ventricle is occasionally present, while concordant ventriculoarterial connections may be seen rarely. Most such hearts have a range of additional abnormalities, including ventricular septal defects; outflow tract obstruction, usually of the morphologically left ventricle; anomalies of the morphologically tricuspid valve; and a highly abnormal location of the specialized atrioventricular conduction axis. Some examples exhibit bizarre abnormalities of ventricular relationships and topology, including criss-cross atrioventricular connections and superoinferior ventricular relations. In describing the anatomy of these malformations, it is important to use a step-by-step segmental approach to the documentation of the connections and associated defects in each case and to avoid potentially confusing shorthand terms.

Aortic translocation, Senning procedure and right ventricular outflow tract augmentation for congenitally corrected transposition, ventricular septal defect and pulmonary stenosis.

The management of congenitally corrected transposition of the great arteries and associated lesions is frequently challenging. Significant pulmonary stenosis is a contraindication to the conventional double-switch. Instead repair may be accomplished by the Rastelli-Senning procedure, using an extracardiac conduit to achieve continuity between the morphological left ventricle and the pulmonary arteries. This however can be accompanied by conduit and intra-ventricular baffle-related problems that can necessitate surgical re-intervention and lead to late mortality. We describe the use of aortic translocation, Senning procedure and reconstruction of the right ventricular outflow tract using autologous tissue and valved homograft to facilitate anatomical correction in congenitally corrected transposition. The advantages of this technique in this group of patients and the implications for conduction tissue are discussed.

The nomenclature, definition and classification of discordant atrioventricular connections.

During the process of creation of a bidirectional crossmap between the system emerging, on the one hand, from the initiative sponsored by the Congenital Heart Committees of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and on the other hand, from that formulated by the Coding Committee of the European Association for Pediatric Cardiology, the Nomenclature Working Group has successfully created the International Paediatric and Congenital Cardiac Code. As would be expected, during the process of crossmapping it became clear that, for most lesions, the European Pediatric Cardiac Code was more complete in its description of the diagnoses, while the International Congenital Heart Surgery Nomenclature and Database Project was more complete in its description of the procedures. This process of crossmapping exemplifies the efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system for the naming of paediatric and congenital cardiac disease, the International Pediatric and Congenital Cardiac Code. Although names and classification for paediatric and congenital cardiac disease will continue to evolve over time, we are now closer than ever to reaching uniform international agreement and standardization. The International Paediatric and Congenital Cardiac Code can be downloaded from the Internet, free of charge, at www.ipccc. net.

Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits.

OBJECTIVES: We sought to evaluate the contribution of unifocalization procedures in the management of patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. METHODS: From 1975 through 1995, 82 consecutive patients were entered in a multistage approach and had 189 sternotomies and thoracotomies to perform 119 shunts, 130 major aortopulmonary collateral artery transplantations, and 76 major aortopulmonary collateral artery ligations. The serial angiographies and the follow-up of these patients were reviewed. RESULTS: The concurrent follow-up rate was 80%. The hospital mortality of the preliminary procedures was 4% (7/189). Fifty-three (65%) patients had a complete repair. The hospital mortality of the repair was 8% (4/53), and 9 late deaths occurred after repair, all of which were cardiac related. The overall survival of all patients to the age of 30 years was 58% +/- 7%. Survival 12 years after complete repair was 51% +/- 14%. On angiography, central shunts promoted growth of central pulmonary arteries in all cases (29 patients). Sixty unifocalized major aortopulmonary collateral arteries were identified in 31 patients. After a mean of 3.2 +/- 4 years, 26 thrombosed, and 12 presented with a stenosis of greater than 50%. Serial measurements of 29 major aortopulmonary collateral arteries showed no signs of growth (P = .25). CONCLUSION: Long-term survival into adulthood of patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries has been achieved with a multistage approach. However, late survival depends exclusively on the growth of the native pulmonary circulation. The few unifocalized major aortopulmonary collateral arteries that did not thrombose failed to grow.

Reconstruction of left main coronary artery with subclavian artery free graft in an infant.

We report the case of a 3-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) with absent left main coronary artery (LMCA). She underwent repair by reimplantation technique with the construction of a short LMCA using two opposite flaps. Two months later she was re-admitted in cardiac failure. Occlusion of the reconstructed LMCA was found by angiogram. At reoperation the right subclavian artery was used as a free interposition graft to reconstruct the LMCA. The post-operative course was uneventful. At 8 months she was asymptomatic and LMCA patency was demonstrated by angiogram.

New approach to multiple ventricular septal defect closure with intraoperative echocardiography and double patches sandwiching the septum.

OBJECTIVE: This was an evaluation of a new approach to the management of multiple muscular ventricular septal defects. The defects were located with epicardial echocardiography, then transfixed with a guide wire inserted directly through the right ventricular free wall. They were closed with a custom-made multilayered double-patch device under cardioplegic arrest through a standard right atriotomy. METHODS: This was a retrospective study of 14 consecutive patients. RESULTS: The median age and body weight at repair were 40 days (range 1 week-8 years, 3 months) and 4.1 kg (2.8-24 kg), respectively. Five patients (36%) had undergone at least one previous sternotomy; 11 patients (78%) had associated cardiac lesions. Closure of the multiple septal defects was successful in 12 patients (85%). Failure to localize all defects led to pulmonary artery banding in 2 patients. One patient had the residual septal defect closed with a percutaneous device 6 months later, and in the second patient the residual defect was closed with a conventional approach 11 months afterward. Two patients had permanent pacemaker insertion. In 279 patient-months of follow-up, there was 1 cardiac arrest on day 1 and no early or late deaths; all children but one are free of cardiac medications, and no significant residual left-to-right shunts were demonstrated in any patient. CONCLUSION: The reported management of multiple ventricular septal defects has been successful in this series, even in neonates and infants with complex associated cardiac lesions. It appears safe, simple, and effective.

Successful stenting for Budd-Chiari syndrome after pediatric liver transplantation: a case series and review of the literature.

Hepatic venous outflow obstruction, known as Budd-Chiari syndrome, is a rare but serious complication of orthotopic liver transplantation and may lead to graft loss. Our aim was to describe a single center experience of percutaneous interventional management of hepatic outflow obstruction post liver transplantation in children. Three children with post-transplant hepatic venous outflow obstruction were treated successfully with percutaneous stent placement. This resulted in long-term resolution of symptoms and was superior to balloon angioplasty both in our series and in cases reported in the literature. Stenting may be the treatment of choice for hepatic venous obstruction after pediatric liver transplantation, particularly if there is a recurrence of stenosis after balloon dilatation.

T wave alternans threshold late after repair of tetralogy of Fallot.

INTRODUCTION: Sustained microvolt-level T wave alternans (TWA) is a marker of increased risk for malignant ventricular arrhythmia. There is a significant risk of arrhythmia and sudden death after repair of congenital heart disease. The aim of this study was to determine the prevalence and characteristics of TWA after repair of tetralogy of Fallot (TOF). METHODS AND RESULTS: TWA was evaluated during bicycle exercise in 49 subjects who had consecutively undergone transatrial-transpulmonary repair. Median values for age, age at repair, and follow-up duration were 14.9 years (11.5-20.8), 1.6 years (0.2-4.9), and 11.6 years (9.4-17.2), respectively. All patients were in New York Heart Association functional class I and were asymptomatic. Median QRS duration was 120 msec (80-150). Sustained TWA was detected in 7 (23%) of 31 subjects with adequate tests. In these 7 subjects, median onset heart rate (HR) was 120 (98-155). Median HR threshold as a percentage of predicted maximum HR (220 - age) was 58% (48-77). Sustained TWA prevalence was not significantly different compared with normal subjects (7/31 vs 9/83; P = 0.1). Onset HR in the TOF group was significantly lower [mean (SD) of 122 (20) vs 139 (12), P < 0.05]. In the TOF group with sustained TWA, the TWA occurred in 4 of 7 at <60% predicted maximum HR versus 1 of 9 normal subjects (P < 0.05); 3 of 7 had onset HR <120 versus 0 of 9 normal subjects (P < 0.03). There was no significant difference in age, gender, transannular patch use, restrictive right ventricular physiology, QRS duration, QTc, QT/QRS dispersion, or nonsustained ventricular tachycardia in subjects with or those without sustained TWA. CONCLUSION: The onset HR for sustained TWA is significantly lower after repair of TOF. Further study is required to determine whether this represents an increased risk for arrhythmia in this patient group.