No abstract

Objective-To determine the early results of balloon expandable stent implantation for aortic coarctation or recoarctation. Design-Prospective observational study. Setting-Two paediatric cardiology tertiary referral centres. Patients-17 patients, median age 17 years (range 4.4 to 45) and median weight 61 kg (17 to 92). Six had native aortic coarctation and 11 had aortic recoarctation; 14 had upper limb systolic hypertension. Of those with recoarctation, eight had had at least one previous balloon dilatation attempt and two of these patients also had further surgical interventions. Intervention-Balloon expandable Palmaz iliac stent implantation. Main outcome measures-Systolic pressures gradients, minimum aortic diameter, upper limb blood pressures, and incidence of aneurysm formation. Results-18 stents were implanted during 18 procedures in the 17 patients. Mean peak systolic pressure gradient fell from 26 mm Hg (95% confidence interval (CI), 21 to 31 mm Hg) before to 5 mm Hg (2 to 8 mm Hg) after stent implantation (p < 0.001), and mean minimum aortic diameter increased from 7 mm (95% CI, 6 to 8 mm) before to 11.3 mm (10 to 12.6 mm) after implantation (p < 0.001). Complications occurred in five patients (bleeding in two, stent migration in two, and aneurysm formation in one). Two patients remained borderline hypertensive and eight were receiving antihypertensive treatment at most recent assessment. Conclusions-Stent implantation for aortic recoarctation and native coarctation gives good immediate results. Careful follow up is necessary to evaluate complications and the long term eVect on blood pressure. (Heart 1999;82:600-606)

Despite some improvements in the outlook for AVSD diagnosed prenatally, the overall prognosis remains considerably poorer than that implied from surgical series. The detection of associated cardiac and extracardiac abnormalities is important in order to give the best indication of the likely outcome when counseling parents.

Primary pulmonary hypertension is a rare disease of childhood, which carries a poor prognosis. Patients often present with severe exercise limitation, and untreated life expectancy is less than 1 year. Pharmacological intervention is directed towards reduction of the raised pulmonary artery pressure with vasodilator treatment, initially with calcium antagonists, although more recently long term prostacyclin treatment has shown benefit in some patients. Heart-lung transplantation remains an option for children with severe disease refractory to therapeutic treatment.A 4 year old Bangladeshi girl with dyspnoea, cyanosis, and signs of a low cardiac output, is described. Initial treatment with prostacyclin was gradually reduced, and maintenance treatment with oral sildenafil (Viagra; Pfizer) instituted. At follow up 3 months later, her exercise capacity was greatly improved and she continues to enjoy a good quality of life without obvious side eVects. In view of the encouraging initial results, this may become an acceptable adjunct in treating this patient group. (Heart 2000;84:e4) Keywords: primary pulmonary hypertension; sildenafil; prostacyclin A 4 year old Bangladeshi girl presented with dyspnoea, cyanosis, and signs of a low cardiac output. She had been seen 18 months before with a cyanotic episode, and had been treated with inhaled agonists for presumed bronchoconstriction. There was no other relevant medical or drug history.Her oxygen saturation was 84% in air, heart rate was 150 beats per minute, and blood pressure 62/40 mm Hg. The pulmonary component of the second heart sound was accentuated, chest was clear to auscultation, and 3 cm of pulsatile liver edge was palpable.ECG showed right atrial enlargement and right ventricular hypertrophy, and chest x ray film showed marked cardiomegaly with normal lung fields. Echocardiography demonstrated a dilated, poorly contracting right ventricle compressing the left ventricle. There was moderate tricuspid valve regurgitation with a Doppler estimated velocity of 5.0 m/s (100 mm Hg) and a systolic blood pressure of 84 mm Hg.She was resuscitated with oxygen and intravenous dobutamine, and prostacyclin at 5 ng/kg/min was infused.

Systemic venous collaterals occur frequently after a bidirectional cavopulmonary anastomosis and are found postoperatively when a significant pressure gradient occurs between cava and right atrium.