Pulmonary Hypertension Caused by Persistent Anomalous Vertical Vein Bridging the Left Subclavian Vein and Left Atrium With Hypertrophic Cardiomyopathy
A 68-year-old woman was admitted to a nearby clinic because of worsening dyspnea with body weight gain and systemic edema. She had had exertional dyspnea for >10 years, and her body weight had increased by 20 kg over the past year. Administration of oral diuretics did not improve her overhydration state. She was referred to our hospital for further treatment. On admission, her heart rate was 66 bpm and blood pressure was 122/80 mm Hg. She showed orthopnea with severe systemic edema. Her arterial oxygen saturation was 88% with room air. An ECG showed 4:1 atrial flatter, and a chest x-ray showed marked cardiomegaly. Her brain natriuretic peptide was 2.718 pg/mL. A transthoracic echocardiogram demonstrated marked concentric hypertrophy of the left ventricle (Figure 1) with severe tricuspid valve regurgitation and preserved ejection fraction. Her estimated right ventricular systolic pressure was 110 mm Hg. An atrial septal defect was not detected. Her mother also had hypertrophic cardiomyopathy. Intravenous administration of diuretics was effective, and her body weight decreased by 10 kg. Subsequent catheterization of the right side of the heart showed pulmonary hypertension (systolic pulmonary artery pressure, 88 mm Hg), and blood gas oxymetry revealed an O2 step-up at the superior vena cava. Her coronary artery system was intact. Histopathology of a biopsy specimen of her left ventricular myocardium demonstrated hypertrophy and mild disarray of cardiomyocytes, which is compatible with hypertrophic cardiomyopathy. A contrast-enhanced dynamic computed tomography scan was useful for detecting the presence of abnormal venous connection and shunt flow. An anomalous left upper pulmonary vein draining into the left subclavian vein and left atrium was detected (Figure 2 and Movie I in the online-only Data Supplement). Catheterization of the right side of the heart was performed again, and a blood oxygen sample test revealed significant left-to-right shunt flow (Qp/Q s shunt ratio, 1.98).
A preoperative balloon occlusion test of the shunt vessel was performed to evaluate the indication of shunt occlusion. A Selecon MP Catheter II, an occlusion balloon catheter, was placed in the persistent vertical vein through a left internal cervical vein puncture, approaching the left atrium. The balloon was dilated within the persistent vertical vein, with left atrial pressure monitored by the catheter tip. The pulmonary artery pressure was monitored with a Wedge Pressure Catheter placed in the main pulmonary artery. The persistent vertical vein was too large with too high a flow to be occluded for more than several seconds. During balloon occlusion (diameter, 20 mm), the left atrial pressure increased markedly from 20 to 30 mm Hg (Figure 3), although there was little change in the pulmonary artery pressure. From these results, it was expected that primary total closure of the shunt could result in uncontrollable pulmonary congestion after the operation.
The patient underwent partial ligation operation of the abnormal connecting vein after stabilization of her condition. After the ligation operation, her symptoms improved from New York Heart Association class III to II, and her pleural effusion decreased markedly (Figure 4). She was hospitalized once with mild heart failure, but otherwise, her postsurgical course was uneventful for about a year after the operation. Two months after the operation, a balloon occlusion test was performed again. Injected contrast dye flowed to and from between the left atrium and left subclavian vein. During balloon occlusion (diameter, 20 mm), the mean pulmonary artery wedge pressure increased from 18 to 21 mm Hg, but the increase was much less than that in the preoperative test. The shunt (circumference, 35 mm; diameter, ≈11 mm) was smaller than before. From these results, we concluded that total occlusion of the shunt was feasible. As soon as Amplatzer Vascular Plugs are approved and become available in our hospital, we are going to perform percutaneous closure of the residual shunt.
Transcatheter treatment of persistent vertical vein is very rare, and there is not a large number of reports available on the differences between transcatheter shunt occlusion and surgery. According to a report on surgical closure of patent ductus arteriosus, the complete success rate is 94% to 100% with a 0% to 2% mortality rate.1 No such data are available on surgical correction of vertical vein. Although surgical banding is often accompanied by more postoperative pain than transcatheter occlusion, the persistent vertical vein is too large and the flow of the shunt is too high for it to be occluded with vascular closure devices in the present case. It is recommended that, as for the Amplatzer Vascular Plug, the device diameter size should be 30% to 50% bigger than the target vessel diameter.2 The largest Amplatzer Vascular Plug II available is 18 mm in diameter, but the shunt vessel diameter is much bigger (20 mm) in the present case. In addition, it is expected to be very difficult to achieve proper partial shunt vessel occlusion with coils or other occlusion devices to prevent uncontrollable pulmonary congestion with a transcatheter procedure. For these reasons, we chose surgical correction of the shunt.
The abnormal bridging vessel in the present case is considered a persistent vertical vein or a rare variation of partial abnormal pulmonary venous return (PAPVR).The vertical vein is also called levoatriocardinal vein.3 PAPVR occurs in ≈0.5% of the general population. In asymptomatic patients, the condition remains undiagnosed until adulthood in most cases. In previous studies on adult cases, the left upper pulmonary vein is the most affected vein, accounting for 47% to 79% of the PAPVR cases. The persistent vertical vein is often confused with a persistent left superior vena cava (PLSVC) connecting to the left atrium. Four important differences have been pointed out in the past reviews. First, PAPVR usually drains blood in a cephalad direction from the left upper lobe to the left innominate vein, but PLSVC drains blood in a caudal direction from the left subclavian and jugular veins to the right atrium. Second, the absence of a left innominate vein is a reliable indicator of PLSVC. Third, in PLSVC, the left superior vena cava and left superior pulmonary vein are present anterior to the left main bronchus, but in PAPVR, no vessel is present in this location. Finally, PLSVC usually drains into the coronary sinus.
One case of a persistent vertical vein connected to the left atrium has been reported to have left-to-right shunt. In that case, the underlying situation was mitral valve stenosis.4 To the best of our knowledge, this is the first reported case of the association of left-to-right shunt via persistent vertical vein and hypertrophic cardiomyopathy.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.114.007639/-/DC1.
- © 2014 American Heart Association, Inc.