THE SURGICAL TREATMENT of most forms of congenital heart disease is a remarkable success story. Mortality figures are low even for complex conditions such as transposition of the great arteries and tetralogy of Fallot. Despite the immediate operative success, however, important residua, sequelae and late complications have been reported in the more common conditions (table 1). In this issue of ...

Fontan circulation is the consequence of an operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. An 18-year old man with a history of congenital heart disease surgically treated with Fontan circulation, presented with pleuritic chest pain and a raised D-dimer level. Perfusion/ventilation SPECT was performed to exclude the possibility of p...

Single ventricle hearts are congenital cardiovascular defects in which the heart has only one functional pumping chamber. The treatment for these conditions typically requires a three-staged operative process where Stage 1 is typically achieved by a shunt between the systemic and pulmonary arteries, and Stage 2 by connecting the superior venous return to the pulmonary circulation. Surgically, t...

Congenital heart disease (CHD) is the most common congenital anomaly, with single ventricle (SV) defects accounting for nearly 10% of all CHD. SV defects tend to be the most severe forms of CHD: all patients born with SV require multiple open heart surgeries, often beginning in the neonatal period, ultimately leading to the Fontan procedure. Due to improvements in surgical procedures and medica...

PURPOSE Adult patients with congenital heart disease presenting for cardiothoracic operation pose special demands for the arrangement of their perioperative care. This study describes the distinctive perioperative setup for adult congenital heart patients. MATERIALS AND METHODS A retrospective review of 48 adult congenital heart disease cases that received operative intervention from July 200...

BackgroundSarcopenia is one of the important predictors heart failure (HF) in patients with cardiac problems. Skeletal muscle pump to maintain Fontan circulation. We aimed quantify volume psoas major (PMM) and investigate whether it relevant HF hospitalization adults circulation.MethodsA total 154 circulation, 30 age-matched were studied retrospectively. identified secondary sarcopenia by measu...

OBJECTIVES Our aim was to examine the prevalence of arrhythmias and identify independent associations of time to arrhythmia development. BACKGROUND Since introduction of the Fontan operation in 1971, long-term results have steadily improved with newer modifications. However, atrial arrhythmias are frequent and contribute to ongoing morbidity and mortality. Data are lacking regarding the preva...

BACKGROUND In acquired heart disease, brain natriuretic peptide (BNP) and N-Terminal pro-brain natriuretic peptide (NT-proBNP) are increasingly used as diagnostic and prognostic markers. In adult congenital heart disease, the abnormal anatomy and physiology complicate assessment of cardiac function. We studied the clinical correlates of measurement of natriuretic peptides (NP) in adults with a ...

BACKGROUND Right heart hemodynamics were analyzed with a catheter-mounted velocity meter in seven patients after direct atrioventricular anastomosis for Fontan procedure (RV group) and were compared with those obtained in eight patients after direct atriopulmonary anastomosis (RA group). METHODS AND RESULTS In the RV group, cardiac output was 2.7 +/- 0.6 l/min/m2; mean right atrial and pulmon...