ABC | Volume 115, Nº1, Suplement, July 2020

Clinicoradiological Correlation Case 4/2020 – Prolonged Time (38 Days) of Bilateral Pleural Effusion after Cavopulmonary Surgery, Relieved by Embolization of Systemic-Pulmonary Collateral Vessels, in a 40-Month-Old Child with Complex Heart Disease Edmar Ati k, R aul Arrieta, Fernando Antibas Atik Hospital Sírio Libanês de São Paulo, São Paulo, SP – Brazil Keywords He a r t De f e c t s , Con g e n i t a l / c i r u r g i a ; Fon t a n Procedure; Double Outlet Right Ventricle; . Heart Septal Defects,Ventricular; Pulmonary Valve Stenosis. Clinical Data The fetal diagnosis of complex cardiac anomaly (Double- outlet right ventricle, severe pulmonary stenosis due to anterior deviation of the infundibular septum, trabecular interventricular septal defect and hypoplasia of the left ventricle and mitral valve) was confirmed shortly after birth with severe hypoxia, relieved by prostaglandin E1 administration and dilation of the ductus arteriosus by percutaneous stent. With the recurrence of more severe hypoxia, the bidirectional Glenn operation was performed at 9 months of age. Good patient evolution was observed up to 39 months, when total cavopulmonary (Fontan) operation was performed due to the recurrence of hypoxia with 70% oxygen saturation. The patient received propranolol and ASA up to the last intervention. Physical examination: good general status, eupneic, marked cyanosis, normal pulses in the 4 limbs. Weight: 16.35 Kgs, Height: 91 cm, BP: 90 x 60 mmHg, HR: 116 bpm, O 2 Sat: 70%, Hg = 15.5 g, Hct = 55%. Precordium: nonpalpable ictus cordis, without systolic impulses. Muffled heart sounds, without murmurs. Nonpalpable liver. Clear lungs. Complementary Examinations Electrocardiogram: Sinus rhythm, with right ventricular overload. Chest x-ray: Cardiac area was normal with a cardiothoracic index of 0.47. The pulmonary vascular network was normal. (Figure 1). Echocardiogram: situs solitus in levocardia, concordant atrioventricular connection and Double-outlet right ventricle connected with the anterior aorta, large interatrial septal defect, unrelated trabecular interventricular septal defect, measuring 8 mm in diameter and 4 mm effective area due to subvalvular tissue protrusion causing turbulent flow and with a 22 mmHg interventricular pressure gradient, normal tricuspid valve and dysplastic mitral valve with thickened and redundant leaflets. The mitral valve chordae tendineae passed through the ventricular septal defect (VSD) towards the pulmonary subvalvular region. The pulmonary valve was thick and small, without anterograde flow, with functional atresia. The aortic valve had a good opening and measured 16 mm, while the ascending aorta measured 17 mm. The right ventricle measured 22 mm and the wall hypertrophy was 7 mm. Biventricular contractility was normal. Cardiac Catheterization: It showed similar pressures (10 mm Hg) in the superior vena cava and the pulmonary arterial tree. The pressure in the atria was 5 mmHg. Angiography in the innominate vein highlighted, in addition to the good connection of the superior vena cava in the right pulmonary artery, a well-developed pulmonary tree without obstructions. The venous return through the pulmonary veins showed good ventricular contractility and well-defined cardiac anomaly. Clinical Diagnosis: Double-outlet right ventricle, severe pulmonary stenosis due to anterior deviation of the infundibular septum, trabecular interventricular septal defect and hypoplasia of the left ventricle and mitral valve, with bidirectional Glenn and marked hypoxia. Clinical Reasoning: There were clinical elements leading to an arterial malposition diagnosis due to the markedly muffled heart sounds. Marked pulmonary stenosis was made evident by the absence of heart murmurs. There was no clinical evidence for the diagnosis of left ventricular hypoplasia because the functional dynamics behaved as in the situation of double outlet right ventricle with interventricular septal defect and pulmonary stenosis. The diagnosis was well established by the echocardiography. Differential Diagnosis: In a hypoxic patient without significant murmur and with muffled heart sounds, a wide range of anomalies are included in the differential diagnosis. The main ones are the transposition of the great arteries, a single right or left ventricle and pulmonary atresia with interventricular septal defect. The diagnosis in these circumstances is always established by echocardiographic images. Conducts: It was known from birth that the most adequate approach would be directed at total cavopulmonary surgery, which became necessary given the progression of hypoxia from the age of three. Preliminary data before the functional corrective surgery presumed a good later evolution. However, the patient evolution showed exaggerated bilateral pleural effusion that lasted 38 days, despite treatment with albumin Mailing Address: Edmar Atik • Private clinic. Rua Dona Adma Jafet, 74, conj.73, Bela Vista. Postal Code 01308-050, São Paulo, SP – Brazil E-mail: conatik@incor.usp.br DOI: https://doi.org/10.36660/abc.20190488 31