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Título

Pentalogy of Cantrell associated with Complex Cyanotic Heart Disease: Heart Team Management of a Challenging Disease

Resumo

Introduction:Pentalogy of Cantrell is a rare entity of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. It was first described in 1958 . Case Description:A baby girl weighing 3,915 g was born from a cesarean delivery to a 35-year-old mother, at 41 weeks of gestation with Apgar score 9/10 on a secondary countryside hospital. It was observed omphalocele and ectopia cordis in the delivery room. The patient developed gradually generalized cyanosis, being referred to the neonatal intensive care unit, requiring tracheal intubation, mechanical ventilation and prostaglandin E2 (PGE2) with improvement of oxygen saturation and hemodynamics. It was performed an echocardiogram which showed Mild Tricuspid Regurgitation, Cor triatriatum in the interior of the left atrium with no obstruction, Dilated Right ventricle with severe hypertrophy, Left Ventricular Ejection Fraction 60%, Pulmonary Valve Atresia, Perimembranous Ventricular Septal Defect of 10mm, Ostium Secundum Atrial Septal Defect of 6mm with bidirectional flow, Patent Ductus Arteriosus in addition to a severe stenosis of left pulmonary artery. The patient was transferred to a tertiary hospital. After a brief discussion with the Heart Team, it was planned to perform an urgent palliative Blalock Taussig (BT) to left pulmonary artery, after that, stop the IV PGE2 and evaluate signs of right pulmonary artery stenosis. The procedure happened with no complications. During the immediate postop care and after stopped IV PGE2 the ventilatory parameters needed to be increased mechanically, so it was accomplish an echocardiogram, which presented no evidences of continuous flow by the left BT shunt and minimum right pulmonary artery flow. On a new discussion, the team chose to perform an interventional heart catheterization and, if necessary, in the same procedure, a PDA – Right pulmonary angioplasty with a drug-eluting stent. During heart catheterization it was evidenced a large PDA with significant stenosis of the proximal segments of the right and left pulmonary arteries, a large and functional left BT shunt without stenosis. With this diagnosis, we performed an PDA – Right pulmonary artery angioplasty with a 4x20mm drug eluting stent with no complications. The patient was discharged on the 8th postoperative day with good recovery. Conclusion:No more than 5% of patients survive with Cantrell Pentology. Heart Team management is very important for the treatment of this patients.

Palavras Chave

Comunicação Interventricular; Pentalogia de Cantrell; Tetralogia de Fallot

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