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LATE PRESENTATION OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION
2235 JACC March 21, 2017 Volume 69, Issue 11
FIT Clinical Decision Making LATE PRESENTATION OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION Poster Contributions Poster Hall, Hall C Friday, March 17, 2017, 3:45 p.m.-4:30 p.m. Session Title: FIT Clinical Decision‐Making: Prevention, Adult Congenital and Congenital Heart Disease Abstract Category: Congenital Heart Disease Presentation Number: 1169-390 Authors: Mohammad Thawabi, Sarah Studyvin, Amer Hawatmeh, Rajiv Verma, Marc Cohen, Newark Beth Israel Medical Center, Newark, NJ, USA
Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect. Undiagnosed cases in adulthood, with hemodynamics favorable for surgical repair, are extremely rare. Case: A 30 year old male presented with exertional dyspnea and episodic peripheral cyanosis that progressed over two years. He was diagnosed with an atrial septal defect (ASD) in early childhood, prior to immigrating to the USA. However, due to social and economic issues, he lost medical follow up.
Physical examination revealed a body mass index of 19 kg/m2, oxygen saturation of 85%, and respiratory rate of 20. He had peripheral cyanosis and clubbing. Cardiac auscultation revealed persistently split S2, accentuated P2, and a grade II systolic murmur at the left sternal border. EKG revealed a right bundle branch block and right atrial enlargement.
Transthoracic echocardiogram (TTE) revealed a large secundum ASD with right to left shunt and supracardiac TAPVC via a dilated vertical vein to a dilated innominate vein. The right atrium was severely dilated. The right ventricle was severely dilated with mildly reduced systolic function and moderately elevated systolic pressure. Cardiac MRI confirmed the diagnoses. Decision‐Making: Surgical palliation was deemed necessary. However, given his age and elevated pulmonary artery (PA) pressure on TTE, a decision was made to proceed with cardiac catheterization to obtain objective hemodynamic measurements. Cardiac catheterization revealed a PA pressure of 42/13/27mmHg, pulmonary vascular resistance of 1.5 Wood units, cardiac index of 2.4L/min/m2, and Qp:Qs of 5:1. In light of the patient’s favorable hemodynamics, elective repair of the TAPVC, primary closure of the ASD, and ligation and division of the vertical vein were successfully done. The patient had an uneventful hospital course and was discharged home. The patient’s functional capacity continued to improve on outpatient follow up.
Conclusions: Diagnosis of TAPVC is rarely delayed until adulthood, and is rarely accompanied with acceptable pulmonary hemodynamics. Surgical correction is necessary to improve function and decrease adverse cardiac events.
FIT Clinical Decision Making LATE PRESENTATION OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION Poster Contributions Poster Hall, Hall C Friday, March 17, 2017, 3:45 p.m.-4:30 p.m. Session Title: FIT Clinical Decision‐Making: Prevention, Adult Congenital and Congenital Heart Disease Abstract Category: Congenital Heart Disease Presentation Number: 1169-390 Authors: Mohammad Thawabi, Sarah Studyvin, Amer Hawatmeh, Rajiv Verma, Marc Cohen, Newark Beth Israel Medical Center, Newark, NJ, USA
Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect. Undiagnosed cases in adulthood, with hemodynamics favorable for surgical repair, are extremely rare. Case: A 30 year old male presented with exertional dyspnea and episodic peripheral cyanosis that progressed over two years. He was diagnosed with an atrial septal defect (ASD) in early childhood, prior to immigrating to the USA. However, due to social and economic issues, he lost medical follow up.
Physical examination revealed a body mass index of 19 kg/m2, oxygen saturation of 85%, and respiratory rate of 20. He had peripheral cyanosis and clubbing. Cardiac auscultation revealed persistently split S2, accentuated P2, and a grade II systolic murmur at the left sternal border. EKG revealed a right bundle branch block and right atrial enlargement.
Transthoracic echocardiogram (TTE) revealed a large secundum ASD with right to left shunt and supracardiac TAPVC via a dilated vertical vein to a dilated innominate vein. The right atrium was severely dilated. The right ventricle was severely dilated with mildly reduced systolic function and moderately elevated systolic pressure. Cardiac MRI confirmed the diagnoses. Decision‐Making: Surgical palliation was deemed necessary. However, given his age and elevated pulmonary artery (PA) pressure on TTE, a decision was made to proceed with cardiac catheterization to obtain objective hemodynamic measurements. Cardiac catheterization revealed a PA pressure of 42/13/27mmHg, pulmonary vascular resistance of 1.5 Wood units, cardiac index of 2.4L/min/m2, and Qp:Qs of 5:1. In light of the patient’s favorable hemodynamics, elective repair of the TAPVC, primary closure of the ASD, and ligation and division of the vertical vein were successfully done. The patient had an uneventful hospital course and was discharged home. The patient’s functional capacity continued to improve on outpatient follow up.
Conclusions: Diagnosis of TAPVC is rarely delayed until adulthood, and is rarely accompanied with acceptable pulmonary hemodynamics. Surgical correction is necessary to improve function and decrease adverse cardiac events.