RESUMO
A case of absent right aortic cusp causing serious aortic incompetence in a neonate is reported. A "bicuspidalization" repair broke down within 24 hours, but reoperation with annular enlargement and insertion of a 19-mm prosthetic valve resulted in complete recovery. This rare but life-threatening anomaly of the aortic valve can be recognized with Doppler echocardiography. Valve replacement in conjunction with aortic annular enlargement should be performed as primary repair.
Assuntos
Insuficiência da Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Insuficiência da Valva Aórtica/etiologia , Humanos , Recém-Nascido , Masculino , ReoperaçãoRESUMO
From 1979 through 1991, 19 infants with absent pulmonary valve syndrome and airway obstruction were seen for surgical treatment. All patients underwent extensive pulmonary artery aneurysmorrhaphy using cardiopulmonary bypass. Fourteen patients had simultaneous transatrial ventricular septal defect (VSD) closure, infundibular resection, and placement of a short transannular patch; 2 had transventricular VSD closure and infundibular resection without a transannular patch; 1 underwent transventricular VSD closure and transannular patching; and 2 underwent pulmonary artery aneurysmorrhaphy alone with the VSD left open. All 19 infants had good hemodynamics when taken from the operating theater, but 3 died postoperatively of severe airway obstruction, despite further tracheobronchopexy procedures in 2 (hospital mortality rate, 16%; confidence limits, 7% to 29%). Among the 16 patients discharged from the hospital, there was one late death. Five other patients have required reoperation for branch pulmonary artery stenosis (n = 2), residual airway obstruction resulting from persistent pulmonary artery dilatation (n = 1), closure of VSD (n = 1), and homograft valve insertion for pulmonary incompetence and right ventricular dysfunction (n = 1). There are 15 long-term survivors. Eight of them have episodic bronchospasm of mild to moderate severity, and all are responsive to sympathomimetic bronchodilator aerosols. The remaining 7 are asymptomatic.
Assuntos
Obstrução das Vias Respiratórias/complicações , Procedimentos Cirúrgicos Cardíacos/métodos , Anormalidades Congênitas/cirurgia , Valva Pulmonar/anormalidades , Obstrução das Vias Respiratórias/mortalidade , Procedimentos Cirúrgicos Cardíacos/mortalidade , Procedimentos Cirúrgicos Cardíacos/normas , Ponte Cardiopulmonar/métodos , Ponte Cardiopulmonar/normas , Anormalidades Congênitas/mortalidade , Feminino , Seguimentos , Mortalidade Hospitalar , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Masculino , Reoperação/estatística & dados numéricos , Taxa de Sobrevida , Resultado do Tratamento , Vitória/epidemiologiaRESUMO
A 10-day-old boy with pulmonary atresia received a right-sided aortopulmonary polytetrafluoroethylene shunt. Three days after the operation he became cyanotic and was reintubated. Shunt occlusion was confirmed with angiography. Recombinant tissue plasminogen activator was given locally into the proximal end of the shunt. The thrombus was completely resolved after 2 days. When administration of recombinant tissue plasminogen activator was stopped, heparin infusion was started for 5 days. Shunt patency was demonstrated by angiography at 3 months postoperatively.