RESUMO
This report presents a case of anatomic repair in a young child with double discordance, ventricular septal defect, and pulmonary atresia. A novel technique of hemi-Senning is described, combined with a bidirectional cavopulmonary anastomosis and Rastelli repair. The possible advantages and applications of this technical modification are briefly discussed.
Assuntos
Anastomose Cirúrgica/métodos , Comunicação Interventricular/cirurgia , Ventrículos do Coração/anormalidades , Atresia Pulmonar/cirurgia , Transposição das Grandes Artérias , Humanos , Lactente , MasculinoRESUMO
Background: Pulmonary arterial hypertension (PAH) is a devastating, life-threatening disease with poor prognosis when left untreated. The long-term prognosis is definitely influenced by the natural progression of PAH but late disease-specific complications may also contribute. Case summary: We present a patient with a long-standing idiopathic PAH in whom progressive dilatation of pulmonary trunk and pulmonary arteries leads to compression of the left main coronary artery and the left atrium with hemodynamic compromise. Conclusion: With the current treatment options, survival in PAH has improved. Guidelines focus on more aggressive treatment with initial combination therapy and earlier referral for transplantation.