Double-outlet left ventricle: A rare case.

Double-outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. DOLV is associated with high mortality, generally due to heart failure, myocardial infarction, or aortic thrombosis. With surgery, the 5-year survival rate is estimated at 70%-75%. Most patients will continue to present with residual cardiac anomalies, such as aortic or mitral valve regurgitation, arrhythmias, or hypertension. Here, we report a 25-year-old male with DOLV with pulmonary stenosis and patent ductus arteriosus who presented to us with hemoptysis, which was due to respiratory tract infection. He improved with standard therapy.

Introduction

A double-outlet left ventricle (DOLV) or a single ventricle is a rare congenital heart disease seen in 5 in 100,000 new born comprising 1% of all congenital heart defects where both aorta and pulmonary artery arise from the left ventricle, and thus, blood is unable to be pumped to the lungs. The right ventricle is, thus, hypoplastic or does not exist. Without life prolonging interventions, this rare condition is fatal, but with interventions, the new born may survive. Mortality is very high but a few patients reach middle age. We present a young male with DOLV who presented to us with features of secondary polycythemia and hemoptysis as a sequelae of posttubercular bronchiectasis.

Case Report

A 25-year-old male presented to us with repeated hemoptysis for 3 days. There was no history of fever, cough, difficulty in breathing, chest pain, or cyanotic spells. His relatives gave a history of recurrent hospitalization in the past for similar complaints. He also had a history of phlebotomy. He was a diagnosed case of DOLV with pulmonary stenosis and a patent ductus for which he was operated in 2000, and a Blalock Taussig shunt was done. There was no history of antenatal exposure to drugs, smoking, or alcohol. It was a full-term normal vaginal home delivery. The patient had poor weight gain during childhood and had a history of easy fatigability. He was not able to play normally like other children. There was also a past history of miliary tuberculosis for which he took a complete course of antitubercular treatment.

On admission, the patient was not dyspneic, had central cyanosis, deeply congested palpebral conjunctiva because of secondary polycythemia, grade 4 clubbing [Figures 1 and 2], midline scar on chest and a pectus carinatum [Figure 3]. The apical impulse was situated in fifth intercostal space 1 cm lateral to midclavicular line and there was a palpable P2. A grade 3/6 ejection systolic murmur best heard in pulmonary area. The JVP was not raised; there was no hepatosplenomegaly, no pedal edema, and/or ascites.

Figure 1

Grade IV clubbing

Figure 2

Grade IV clubbing

Figure 3

Photograph of patient showing a midline scar and pectus carinatum

As the patient presented with hemoptysis and there was no evidence of infection or any signs of pulmonary edema, he was started with supportive treatment with Inj. Vitamin K and IV ethamsylate. Meanwhile, the patient was being investigated and the blood reports revealed: Hb - 18 g%, TLC – 9,500/cumm, and platelets - 1.5 lacs. His liver, renal function tests, and serum electrolytes were normal. Serum calcium was 9.67 mg%, serum inorganic phosphorus 4.18 mg%, serum uric acid 11.8 mg%, and INR was 1.37. Chest X-ray showed right upper and middle zone opacity along with cardiomegaly. Ultrasonography of abdomen showed enlarged nodes at splenic hilum, hepatomegaly, and renal parenchymal disease. Computed tomography thorax showed mediastinal adenopathy and bronchiectatic changes with nonhomogenous opacities in the same area as the chest X-ray. ECG showed a coronary sinus rhythm with left ventricular hypertrophy [Figure 4]. Two-dimensional echocardiography was done which showed left ventricular hypertrophy with both aorta and pulmonary artery arising from left ventricle with a rudimentary right ventricle [Figures 5 and 6]. As there was no evidence of infection, a diagnosis of secondary polycythemia with hyperuricemia with post-tubercular bronchiectasis was made. As the primary mode of management of polycythemia is to decrease the hemoglobin levels to prevent further complications, phlebotomy was done. This involved a bloodletting of 450 mL via the brachial vein and infusing equal amount of 10% dextrose over 1 h to make up for the loss in blood volume. Meanwhile, he was also receiving drugs such as tranexamic acid and IV ethamsylate as styptic agents to control the hemoptysis and febuxostat 40 mg once a day for hyperuricemia. The Hb decreased to 14.7 g% and the patient was discharged on tranexamic acid 500 mg qid and febuxostat.

Figure 4

Electrocardiogram showing a coronary sinus rhythm with left ventricular hypertrophy

Figure 5

Echocardiography images 1 and 2 showing the origin of pulmonary and aorta from left ventricle

Figure 6

Echocardiography image 3 and 4 showing left ventricular hypertrophy with a rudimentary right ventricle

Discussion

DOLV is a rare congenital cardiac malformation in which both great arteries originate entirely or predominantly from the morphologic left ventricle and is associated with a high mortality and guarded outcomes from residual lesions. The diagnosis can be made in uterus by echocardiography and if the gestational age is <24 weeks pregnancy should be terminated.[1] It should be differentiated from similar looking conditions on echocardiography such as a large VSD, atrioventricular canal, double-outlet right ventricle.[2] Mortality is very high (85%) in first 2 years of life but decreases to 9% between 2 and 15 years. Few reach the middle age. In our case, the young man with DOLV with a pulmonary stenosis with a patent ductus arteriosus, so the prognosis was better and the development of pulmonary hypertension was late. Hemoptysis was mainly attributed to post-tubercular bronchiectasis. The most common complications of DOLV include clubbing, congestive heart failure, frequent pneumonias, and arrhythmias.[345] In congenital cardiac lesions, hemoptysis is not necessarily related to a problem in the heart (pulmonary hypertension and anastomotic leaks). The cause could perhaps lie in the lungs and one might have to investigate in detail to locate the source of bleed, which will help in initiating appropriate therapy.

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Conflicts of interest

There are no conflicts of interest.