Double-Outlet Right Ventricle with Dextrocardia in a One-year-old girl: A Case Report

Author : D. Hertriwibowo, K. N. Hapsara, F. S. Hasibuan
Upload Date : 19-04-2018

Background: Double outlet right ventricle (DORV) is a heterogeneous collection of lesions that share one morphologic feature, namely that both the aorta and the pulmonary artery arise from the right ventricle. It is a very rare case and only comprises about 1-1,5% of all congenital heart diseases.

Methods: We report a rare case of DORV with dextrocardia in a one-year-old girl.

Results: A one-year-old girl, presented to pediatric outpatient clinic of  Dr. R. Koesma General Hospital, with chief complaint of cough followed by dyspnea. She had history of recurrent cough, breathlessness and cyanosis that occurred when she was crying. Upon examination, no cyanosis was found. Harsh systolic murmur was heard on the left sternal border. Chest x-ray revealed dextrocardia and decreased pulmonary vascular markings. The patient was diagnosed with congenital heart disease (CHD) and then consulted to cardiologist for echocardiography evaluation to confirm the diagnosis.

2D echocardiography showed the following findings: situs solitus; dextrocardia mirror image; critical pulmonary stenosis with VA connections discordance; Aorta overriding more than 50%, ventricular septal defect malalignment; PDA with left to right shunt and pulmonary artery arise from the right ventricle suspecting DORV.

The patient was then referred to Dr. Soetomo Hospital for further evaluation and treatment.

Conclusion: Despite the rarity of DORV, physician needs to be aware of this disease, as the clinical presentation can be vary. This congenital heart disease is almost always associated with VSD, and in some cases, it can also be accompanied by other cardiac anomaly, which will later affect the clinical presentation.

We can see in this case report that echocardiography plays an important role for diagnosing this disease with all the cardiac anomaly, thus helps determining the right treatment and intervention for the patient.

KEYWORDS : DORV, echocardiography, congenital heart disease, one-year-old girl.

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