The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair.

The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.

Objective: To determine if in-hospital mortality after cardiac surgery can be predicted, in children, using a new clinical and surgical index. Study design: Observational, retrospective, cross-sectional. Methods: We reviewed 818 charts from children undergoing surgery between January, 2000, and December, 2004. The index was calculated by summing the scores from five variables, specifically age, nutritional state, the presence of associated clinical risk factors, surgical complexity, and use and time of cardiopulmonary bypass. Each variable was subdivided into categories of low, medium or high risk, with scores attributed as zero, one or two, respectively. Risks for death were calculated using the odds ratio. Results: Our overall mortality was 14.7%, with our proposed index correlating strongly with mortality (p less than 0.0001). No patients died with scores of zero, but mortality increased from around 10% with a score of three, to close to 30% with scores of five and six, and to over 50% with a score of eight. No patients reached scores of 10, and more than three-fifths of all patients had scores between zero and three. We observed higher mortalities independently for each variable in association with the highest risk scores. Conclusions: We found that surgery undertaken in the neonatal period, weight below the 5th percentile, the presence of associated clinical risk factors, operations of higher complexity, and more than 90 minutes of cardiopulmonary bypass were all significantly associated with mortality. Our suggested new index showed a linear correlation with mortality, and in our current experience, has proved a valuable tool for predicting adverse outcomes.

Objective: To establish the feasibility and efficacy of closing large patent arterial duct in infants, using the modified and angled variants of the Amplatzer duct occluder. Background: Closure of large patent arterial ducts by inserting devices in sick and underweight infants, particularly those weighing around 5 kilograms, remains a challenge. Bigger devices require larger delivery sheaths and may cause obstruction either to the aorta or left pulmonary artery. Negotiating a large device is difficult or impossible, as the sheath gets kinked. Because of these problems, such underweight infants with large ducts who are failing to thrive, and in left ventricular failure with associated lesions, are typically referred for surgery, often leading to higher morbidity and mortality. Methods: We attempted to close such large patent arterial ducts using the new Amplatzer occluder, modified with single layer of polyester, and the angled occluder, with no polyester material, inserted through a specially braided kink-resistant sheath. Results: Closure was achieved in 10 infants, with mean age of 8.2 months, mean weight of 5.5 kilograms, the lowest weighing 3.9 kilograms. The mean size of the patent ducts was 6.3 millimetres, with the largest measuring 8.6 millimetres. We implanted 6 modified and 4 angled occluders. In one patient, suffering from hydronephrosis, a 14/12 angled device embolized and was retrieved, but the patient died. In the remaining patients, all ducts were closed completely, with no obstruction to either the aorta or left pulmonary artery. On follow up, all showed excellent clinical improvement. Conclusion: Complete closure of very large patent arterial ducts is now possible, even in very sick and underweight infants, using the large but low profile custom-made angled or modified versions of the Amplatzer occluder.

Background: Surgical intervention for children with congenital cardiac disease in the developing world often occurs late. Our objective was to identify factors that placed Guatemalan children at risk for delayed care. Methods: We investigated the medical and socioeconomic background of 178 children under the age of 18 years who received their first corrective surgery for congenital cardiac disease at the Unidad de Cirugía Cardiovascular de Guatemala in 2002. A retrospective review of medical records was performed. Each case was stratified into one of three surgical classes based upon customary practice in the United States of America. The outcome we measured was age at surgery, adjusting for the surgical class. Logistic regression was performed and odds ratios calculated. Results: In univariate analyses, patients presented later for surgery if they were from rural areas (p equals 0.001), did not have social security membership (p equals 0.004), or paid any amount towards the cost of their surgery (p less than 0.001). Age at surgery was also positively correlated with the distance of the home of the patient from the surgical centre (p equals 0.002). For the subset of patients who applied for financial assistance, we found that children presented later for surgery if they required institutional support (p equals 0.001), or came from households of larger size (p less than 0.001). Conclusions: Guatemalan children with congenital cardiac disease may be at risk for delayed surgical care if they come from rural areas, areas distant from the surgical centre, or from families without membership of social security.

It is uncommon for thrombus to form within the heart of neonates with congenital cardiac disease. We describe a newborn with coarctation of the aorta, in whom a left atrial thrombus was discovered on the second day of life, and was thought to have been present before birth.

Tissue Doppler measurements of the right and left lateral ventricular walls were made before and after perforation of the pulmonary valve using radiofrequency energy in a patient with pulmonary atresia and intact ventricular septum. The ratio of peak tissue velocity during rapid ventricular filling to atrial contraction increased for both atrioventricular valves after perforation of the pulmonary valve, and the patient was able to be weaned off prostaglandins without further intervention. Such measurements made using tissue Doppler may aid in the management of patients with pulmonary atresia and intact ventricular septum by predicting improvements in right ventricular relaxation.

A newborn girl with atresia of the common pulmonary vein, presented immediately after birth with severe cyanosis and acidosis. The diagnosis of totally obstructed total pulmonary venous return was made by cross-sectional echocardiography. Subsequent cardiac catheterization failed to demonstrate the site of pulmonary venous return. Necropsy showed the pulmonary veins to be connected bilaterally to an atretic common pulmonary vein. There was no obvious alternative pathway for pulmonary venous return.