SIGNIFICANCE OF PULMONARY VENOUS OBSTRUCTION IN TOTAL ANOMALOUS PULMONARY VENOUS RETURN

Ping-Yao Wang¹, Be-Tau Hwang¹, Ming-Chih Tsai¹, Jen-Her Lu¹, Chui-Mei Tiu²,

Zeng-Chung Weng³and Luara C.C. Meng¹

Department of Pediatric Cardiology¹, Radiology² and Cardiovascular Surgery³, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine ⁴, Taipei, Chinese Taipei

Background: Total anomalous pulmonary venous return (TAPVR) is a less common congenital cardiovascular anomaly and poor natural prognosis. It has been detected more frequently in recent year due to the advent of echocardiography and the cardiovascular magnetic resonance imaging. (MRI)

Object: The aim of this study was to evaluate the clinical manifestations and outcomes in patients of with and without pulmonary venous obstruction.

Materials and methods: Between January 1985 though December 2000, a total of 27 cases with TAPVR were enrolled for retrospective analysis. All of them had received two-dimensional and color Doppler echocardiography examination. Twenty-six of them had been cardiac catheterized for diagnosis, and the other one who died at age of one day had only echocardiographic diagnosis. Of them, 23 had been operated and 4 didn’t received operation including three died before operation and the other one with complex heart disease. In addition, 10 of 27 cases had cardiovascular MRI examination for the further study. Data was analyzed by z-test and Chi-square test for obstructed and non-obstructed TAPVR categories. Statistical significance was obtained if p<0.05.

Results: Among the 27 cases, 15 (56%) patients had pulmonary venous obstruction and 12 (44%) patients had no pulmonary venous obstruction. There were not significant difference of sex and pulmonary venous drainage sites in those two groups. There were isolated TAPVR in 20 cases and complicated in 7. Pulmonary venous obstruction was noted in 11 (55%) of isolated TAPVR and 4 (57%) of non-isolated TAPVR. Pulmonary hypertension was present in 18(69%) of 26 cases who had received cardiac catheterization. Among them, 10 had pulmonary venous obstruction and 5 had systemic level of pulmonary arterial pressure. Cyanosis was the obviously clinical symptom in obstructed TAPVR (80% v.s. 30%, p=0.038). Seven (30%) of 23 patient who had received operation died, however, three (75%) of 4 patients without operation expired. There was no significant difference of surgical mortality between obstructed and non-obstructed groups by age categories. (33% v.s. 27%)

Conclusion: Cyanosis is the obviously clinical symptom of obstructed TAPVR. There is no significant difference of surgical mortality by age between obstructed and non-obstructed groups. Early detection and surgical treatment for TAPVR are important. Although cardiac catheterization and angiocardiography is the golden standard for the diagnosis, 2-D and color Doppler echocardiography has been provided the quick and accurate diagnostic image of TAPVR.

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