Hot topics in tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "hot topics" to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%.

Warfarin Overdose in a Breast-feeding Woman.

We describe a case of a breastfeeding woman with an accidental warfarin overdose resulting in a markedly elevated prothrombin time. The breast-fed infant was evaluated and tested for ill effects. We discuss the use of warfarin while breast-feeding.

Outcome of pulmonary valve replacements in adults after tetralogy repair: a multi-institutional study.

Objective: The purpose of this study was to assess the outcome of pulmonary valve replacement (PVR) in adults with moderate/severe pulmonary regurgitation after tetralogy repair, with particular emphasis on patient outcome, durability of valve repair, and improvement in symptomatology.

Design/setting/patients: The project committee of the International Society of Congenital Heart Disease undertook a retrospective multi-institutional analysis of PVR. Seven centers participated in submitting data on 93 patients >18 years of age who had the operation performed and follow-up obtained.

Dosing of clopidogrel for platelet inhibition in infants and young children: primary results of the Platelet Inhibition in Children On cLOpidogrel (PICOLO) trial.

Background: Infants and young children with certain types of heart disease are at increased risk for thromboses. Clopidogrel 75 mg/d is used in adults to prevent thrombotic events. The dose to achieve similar platelet inhibition in children is unknown.

Congenitally corrected transposition of the great arteries: an update.

Congenitally corrected transposition of the great arteries is a rare condition in which systemic venous blood returns to normally positioned atria. However, the atria are connected to the opposite ventricle, right atrium to left ventricle, left atrium to right ventricle; so-called atrioventricular discordance. In addition, the ventricles are inverted (right to left change in position) and are connected to the opposite great artery, left ventricle to pulmonary artery, right ventricle to aorta; thus, forming ventricular-arterial discordance.

Clinical outcomes of palliative surgery including a systemic-to-pulmonary artery shunt in infants with cyanotic congenital heart disease: does aspirin make a difference?

Background: Aspirin (ASA) often is used to prevent thrombosis in infants with congenital heart disease after placement of a systemic-to-pulmonary artery shunt, but its effect on outcomes is unknown.

Methods And Results: The present multicenter study prospectively collected data on 1-year postoperative rates of death, shunt thrombosis, or hospitalization age <4 months for bidirectional Glenn/hemi-Fontan surgery in 1004 infants. The use and dose of ASA were recorded.

Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease.

A member of the Tennessee state legislature recently proposed a bill that would mandate all newborn infants to undergo pulse oximetry screening for the purpose of identifying those with critical structural heart disease before discharge home. The Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Defects was convened on September 29, 2005. This group reviewed the current medical literature on this topic, as well as data obtained from the Tennessee Department of Health, and debated the merits and potential detriments of a statewide screening program.

Pregnancy outcomes after atrial repair for transposition of the great arteries.

Increasingly, women born with complete transposition of the great arteries who have undergone atrial repair by either the Senning or the Mustard procedure are reaching childbearing age. This study reports on pregnancy outcomes after the atrial repair of transposition of the great arteries. Record review and standardized questionnaires were used to ascertain the outcomes of 70 pregnancies reported in 40 women (36 Mustard procedures, 4 Senning procedures).

Clinical presentations of unoperated and operated adults with congenital heart disease.

The clinical presentation of adults with congenital heart disease (ACHD) is exceptionally diverse. Operated, unoperated acyanotic, and cyanotic patients often have similar physiologic characteristics, and thus shared clinical symptomatology. Five illustrative cases are included to highlight important considerations in the diagnosis and management of ACHD.

Occlusion of the coronary sinus ostium: a danger for cavopulmonary anastomosis.

Coronary sinus (CS) ostial obstruction decompressing through a left superior vena cava is a rare finding and presents unique risks to patients requiring a Glenn anastomosis for single-ventricle palliation. Preoperative diagnosis is imperative in order to avoid inadvertent ligation or coil occlusion. We report three cases with single-ventricle physiology and CS obstruction identified at cardiac catheterization.