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01.12.2014 | Case report | Ausgabe 1/2014 Open Access

BMC Cardiovascular Disorders 1/2014

Partial anomalous pulmonary venous connection to superior vena cava that overrides across the intact atrial septum and has bi-atrial connection in a 75-year-old female presenting with pulmonary hypertension

Zeitschrift:
BMC Cardiovascular Disorders > Ausgabe 1/2014
Autoren:
Hong Wang, Hanxiong Guan, Dao Wen Wang
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1471-2261-14-149) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

DWW contributed to the conception of the report and revision of the report critically for important intellectual content. HW drafted the manuscript, was responsible for acquisition and interpretation of the data, searched the literature, and revised the figures. HXG performed the MCTA examination, reconstructed the images and drew the figures. All authors were involved in the patient’s treatment and made a substantial contribution to the manuscript preparation. All authors read and approved the final manuscript.

Abstract

Background

Partial anomalous venous connection (PAPVC) is a rare congenital heart disease where the blood flow from one or more pulmonary veins (but not all) returns to the right atrium or systemic venous circulation and is often associated with a sinus venosus atrial defect (SVD). Transthoracic echocardiography (TTE) can provide limited information for this anomaly and the diagnosis of this congenital defect has been a clinical challenge.

Case presentation

We report here a case of a 75-year-old female with adult-onset pulmonary arterial hypertension (PAH), hypoxemia and right-sided chamber dilatation. The diagnosis of PAPVC was made incidentally by multidetector computed tomographic angiography (MCTA) that was performed to exclude pulmonary embolism. In this type of PAPVC, the atrial septum is intact, the right upper pulmonary vein (RUPV) connects to the superior vena cava (SVC), and the SVC overrides across the atrial septum and has bi-atrial connection, all of which are clearly manifested by MCTA.

Conclusions

This case indicates the need to exclude a PAPVC and SVD in unexplained pulmonary hypertension, and MCTA is a reliable non-invasive imaging technique with high resolution and wide anatomic coverage. The case also demonstrates that the coexisting SVD with PAPVC is an anomalous venous connection instead of atrial septal defect (ASD) and its key feature is the overriding of SVC or IVC across the intact atrial septum.
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