Isolated aortic coarctation: experience in 100 consecutive patients.

INTRODUCTION: Coarctation of the aorta (CoA) is a stenosis usually located in the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and presents excellent immediate results but significant residual problems often persist. OBJECTIVES: To describe the presentation, treatment and long-term evolution of a population of 100 unselected consecutive patients with isolated CoA in a single pediatric cardiology center. METHODS: This was a retrospective study of all patients with isolated CoA treated during4 the last 21 years (1987-2008). RESULTS: The patients (n=100, 68.3% male) were diagnosed at a median age of 94 days (1 day to 16 years). The clinical presentation differed between patients aged less or more than one year, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (88 and 63%, respectively; p < 0.01). Treatment, a median of 8 days after diagnosis, was surgical in 79 cases (20 end-to-end anastomosis, 31 subclavian flap, 28 patch) and percutaneous in the remaining 21 (15 balloon angioplasty, 6 with stenting). The mean age of surgical patients was younger than in those treated percutaneously (3.4 vs. 7.5 years; p < 0.01). Immediate mortality was 2% and occurred in the surgical group. There was no late mortality, in a mean follow-up of 7.2 +/- 5.4 years. Recoarctation occurred in 8 patients (6 surgical, 2 percutaneous). There are 46 patients who currently have hypertension (19 at rest, 27 with effort), their median age at diagnosis being older than the others (23 vs. 995 days; p < 0.01). CONCLUSIONS: Isolated CoA has an excellent short-term prognosis but a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment. Its potentially insidious presentation requires a high level of clinical suspicion, femoral pulse palpation during physical examination of newborns and older children being particularly important. Delay in treatment has an impact on late morbidity and mortality. Taking into account the data currently available on late and immediate results, the final choice of therapeutic technique depends on the patient's age, associated lesions and the experience of the medical-surgical team. Hypertension should be closely monitored in the follow-up of these patients, as well as its risk factors and complications.

Percutaneous pulmonary valve implantation: initial experience.

INTRODUCTION: Patients with congenital heart disease who undergo surgical implantation of a conduit between the right ventricle and the pulmonary artery (RV-PA conduit) may develop stenosis and/or insufficiency over time. These cases, which are associated with arrhythmias, RV dysfunction and sudden death, remain a challenge for surgical re-intervention, due to its complexity and associated morbidity and mortality. Percutaneous pulmonary valve implantation (PPVI) is therefore a valid alternative. OBJECTIVE: To report our center's initial experience with PPVI. METHODS: Prospective assessment of clinical, echocardiographic, magnetic resonance, hemodynamic and angiographic data from our series of PPVI. RESULTS: Six patients in NYHA functional class +/- II underwent PPVI. All had significant conduit dysfunction and five had stenosis. The procedure consisted of implanting a bare metal stent to reduce the risk of fracture of the Melody valved stents (Medtronic) that were then successfully deployed in all. The immediate hemodynamic results showed a reduction in RV pressure (94 +/-27 to 44 +/- 7 mmHg), RV/LV pressure ratio (94 +/-27 to 44 +/- 7%) and conduit gradient (65 +/- 28 to 11 +/- 4 mmHg), and no insufficiency. The median duration of the procedure was 180 minutes, with no major complications. Patients were discharged a median of two days after the procedure. After a median follow-up of 7.8 months, patients are in functional class I (5) or II (1), with no evidence of conduit dysfunction on non-invasive assessment. CONCLUSIONS: Our results are similar to the excellent results reported in larger series. PPVI is a valid therapeutic option in patients with conduit dysfunction.