Short-and mid-term outcomes of transcatheter intervention for critical pulmonary stenosis and pulmonary atresia with intact ventricular septum in neonates / 中华儿科杂志

<p><b>OBJECTIVE</b>To assess the safety and efficacy of transcatheter intervention for critical pulmonary stenosis (CPS) and pulmonary atresia with intact ventricular septum (PA/IVS) in neonates.</p><p><b>METHOD</b>From June 2006 to September 2011, 27 neonates including CPS in 19 patients and PA/IVS in 8 patients underwent transcatheter intervention. All patients had membranous stenosis or atresia without severe Ebstein's anomaly and severe right ventricle and pulmonary valve hypoplasia, without right ventricle-dependent coronary circulation in PA/IVS. The mean age was (16.8 ± 9.9) d. The mean weight was (3.3 ± 0.5) kg. Two of them were premature neonates, the weight was 2.3 kg and 2.5 kg, respectively. The procedural success, early outcome, complication rates, midterm results and pulmonary regurgitation were retrospectively studied.</p><p><b>RESULT</b>Twenty-six patients were successfully treated with transcatheter intervention. Right ventricular pressure fell from (112.0 ± 21.0) mm Hg (1 mm Hg = 0.133 kPa) to (50.4 ± 15.9) mm Hg (P < 0.001). The ratio of right ventricular pressure and aortic pressure fell from 1.7 ± 0.1 to 0.7 ± 0.3 (P < 0.001). One patient died early of PA/IVS. Complication occurred in 5 patients. Hemopericardium occurred in 3 patients, tachyarrhythmia in 2 patients. Five patients needed prolonged prostaglandin E(1) infusion for 3 to 14 days because of desaturation after the procedure. No patient needed surgery in neonatal period. At a mean follow-up of (33.5 ± 18.3) months (from 6 months to 5 years), 21 patients had no further transcatheter or surgical intervention. Four patients with CPS had moderate to severe residual pulmonary stenosis after the procedure, 3 of them underwent a second balloon dilation at 3 months of follow-up, the other one was waiting for the second balloon dilation. One patient with PA/IVS was waiting for a bidirectional Glenn procedure because of chronic right ventricular failure. Mild pulmonary regurgitation occurred in 18 patients (69.2%), and moderate pulmonary regurgitation in 8 patients (30.8%).</p><p><b>CONCLUSION</b>Transcatheter intervention for CPS and PA/IVS in neonates is safe and effective. It can avoid neonatal surgery. Some patients may require repeat balloon valvuloplasty in infant period. In most patients surgical or transcatheter intervention could be avoided and mild pulmonary regurgitation was the common finding in midterm follow-up.</p>

Diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To summarize the experience in diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy (ARVC) in children.</p><p><b>METHODS</b>A total of 14 children (7 females and 7 males) with ARVC were involved. The cases underwent electrocardiography, echocardiography, cardiac CT or MRI examinations. All cases were treated with sotalol or amiodarone in combination with propranolol. In 2 cases with drug-refractory ventricular extrasystoles, catheter ablation treatment was performed. In 6 cases with obvious impaired ventricular function, additional pharmacological therapy including vasodilators, diuretics, and digitalis were given.</p><p><b>RESULTS</b>Ventricular extrasystoles occurred in all 14 cases and ventricular tachycardia in 8 cases. Ten cases showed Epsilon wave on electrocardiography. All 14 cases had enlarged right ventricle and reduced right ventricular ejection fraction. CT or MRI examination showed right ventricular dilatation and a thinned wall of right ventricle in 10 cases. Ventricular extrasystoles or tachycardia disappeared in 7 cases and was reduced in 4 cases after treatment. The two children receiving catheter ablation treatment did not present ventricular extrasystoles or tachycardia in a 3-month follow-up. The heart function was improved in the 6 children with obvious impaired ventricular function after pharmacological therapy.</p><p><b>CONCLUSIONS</b>The clinical manifestations are diverse in children with ARVC. A definite diagnosis of ARVC should be based on a combination of electrocardiography and echocardiography examinations. Pharmacological therapy is effective partially. Catheter ablation treatment appears to be a promising option in patients with drug-refractory ventricular extrasystoles.</p>