SESSION TITLE: Pulmonary Vascular Disease Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 27, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: PAPVR is a rare congenital anomaly in which one or more of the pulmonary veins drain into the right atrium or into the systemic venous circulation. The reported incidence is 0.4-0.7% in autopsy series with anomalous drainage from the left lung representing only 10% of the reported cases. (1)
CASE PRESENTATION: A 37-year-old female presented with a gradual onset of exertional dyspnea. The echocardiogram suggested pulmonary hypertension (PH). The patient was referred to our center for further evaluation. Subsequently, right heart catheterization revealed normal pressures, however, the superior vena cava saturation was 95.4% consistent with left to right shunt. The patient underwent chest computed tomography with contrast that demonstrated near complete anomalous pulmonary venous return on the left. The left pulmonary vein drained into the left innominate vein at the upper mediastinum. The left lung had only one left lower lobe segmental branch that drained into the left atrium. (Fig.1) Both right-sided pulmonary veins had normal drainage into the left atrium. Right atrial and right ventricular dilatation were noted. Calculated Qp/Qs ratio was 1.9. Based on NYHA functional class III, right sided dilation, and elevated Qp/Qs ratio the patient was referred for surgical repair.
DISCUSSION: PAPVR with intact atrial septum is a rare cause of left to right shunt in adults. It results from early atresia of the left or right parts of the common pulmonary vein before disappearance of the primitive pulmonary systemic venous connections. (1) The decision making about surgical repair in setting of PAPVR without ASD is important. Patients with isolated PAPVR of a part of one lung who are asymptomatic with no evidence of right sided dilation and Qp/Qs ratio <1.8 can be treated conservatively without developing symptoms. Other patients who are symptomatic, have evidence of right sided dilation, and elevated Qp/Qs ratio >1.8 are likely to develop PH and should be considered for surgical intervention. (2)
CONCLUSIONS: In adults with isolated PAPVR without ASD the decision about surgical repair depends on patient’s symptoms, right heart dysfunction, and presence of significant left-to-right shunt.
Reference #1: Senocak F, Ozme S, Bilgiç A, Ozkutlu S, Ozer S, Saraçlar M. Partial anomalous pulmonary venous return: evaluation of 51 cases. Jpn Heart J. 1994; 35(1):43-50.
Reference #2: Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB. Kirklin/Barratt-Boyes cardiac surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003, p.715-751.
DISCLOSURE: The following authors have nothing to disclose: Karim El-Kersh, Ehab Haj Ali, J Shaun Smith
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