Double outlet Right Ventricle

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Research Letter

Double Outlet Right Ventricle with Unrelated Ventricular Septal Defect and Pulmonary Stenosis, in Natural Evolution, in a 36-Year-Old Woman

Introduction

The congenital defect, characterized by the exit of both large arteries from the right ventricle (RV), completely or even when overlapping more than 50% of one of the arteries over the interventricular septum, presents variable relationships between these arteries and the ventricular septal defect (VSD), as well as association with other anatomical and functional variables.

In the most common associated defect, the isolated VSD, in a subaortic position or in that unrelated to the large arteries, the functional condition is expressed with a predominance of volume overload of the heart as a whole, increased by pressure overload, causing early heart failure.

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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 Atik et al. Raul Arrieta Fernando Antibas Atik

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 mm Hg, HR: 116 bpm, O₂Sat: 70%, Hg = 15.5 g, Hct = 55%.

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Clinicoradiological Correlation

Case 1 / 2019 – Natural Evolution of Double Outlet Right Ventricle with Noncommitted Ventricular Septal Defect and Pulmonary Stenosis in a 28-Year-Old Asymptomatic Woman

Edmar Atik et al. Alessandra Costa Barreto Maria Angélica Binotto

Clinical data

The patient evolved without symptoms from birth, when the diagnosis of heart disease was made, as evidenced by heart murmur. Two years ago, supraventricular extrasystoles caused the use of atenolol, propafenone, and magnesium, without improvement. Infective endocarditis was effectively treated 10 years ago. The patient uses levothyroxine 50 mcg for hypothyroidism.

Physical examination: good overall status, eupneic, acyanotic, normal pulses in the four limbs. Weight: 60 Kg, Height 160 cm, right upper extremity blood pressure: 120 x 70 mmHg, HR: 60 bpm.

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Double Outlet Right Ventricle with Unrelated Ventricular Septal Defect and Pulmonary Stenosis, in Natural Evolution, in a 36-Year-Old Woman

Introduction

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

Clinical Data

Case 1 / 2019 – Natural Evolution of Double Outlet Right Ventricle with Noncommitted Ventricular Septal Defect and Pulmonary Stenosis in a 28-Year-Old Asymptomatic Woman

Clinical data

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Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)

Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)