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Case Reports
. 2010 Dec;19(118):340-4.
doi: 10.1183/09059180.00007610.

Sinus venosus atrial septal defect in a 31-year-old female patient: a case for surgical repair

M A Gatzoulis  1 , G Giannakoulas
Affiliations
Case Reports

Sinus venosus atrial septal defect in a 31-year-old female patient: a case for surgical repair

M A Gatzoulis et al. Eur Respir Rev. 2010 Dec.

Abstract

A 31-yr-old female patient previously diagnosed with idiopathic pulmonary arterial hypertension (PAH) was referred to our centre for further evaluation. Cardiac magnetic resonance imaging (MRI) and echocardiography revealed a superior sinus venosus atrial septal defect (ASD) with partial anomalous pulmonary venous drainage. Following re-diagnosis, surgical repair was considered. Despite a disproportionately high mean pulmonary artery pressure (P(pa)) of 47 mmHg relative to the patient's age and defect, the decision to operate was based on the absence of oxygen desaturation (either at rest or during exercise), cyanosis or abnormally elevated haemoglobin. Other operability criteria included normal sinus rhythm at rest, vasoreactivity and a pulmonary to systemic blood flow ratio of 1.9 at rest. Surgical repair and continued advanced therapy with bosentan 125 mg b.i.d. and aspirin 75 mg o.d. proved successful, with post-operative improvements in exercise capacity and dyspnoea, and a reduction in P(pa) to 25 mmHg. Keen to start a family, the risks of pregnancy were discussed. This case illustrates the importance of secondary PAH in sinus venosus ASD and the need to exclude a sinus venosus ASD in unexplained right ventricular dilatation. Access to expertise in congenital heart disease and use of cardiac MRI can improve diagnosis and, in turn, treatment decisions.

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Conflict of interest statement

Statement of Interest

M.A. Gatzoulis has served the advisory board of Actelion UK, Pfizer and GSK, and has received unrestricted educational funds from Actelion UK and Pfizer. G. Giannakoulas has been involved in a scientific advisory board for Actelion and in addition has lectured for Actelion.

Figures

Figure 1.
Figure 1.
Cardiac magnetic resonance imaging demonstrates the anomalous right upper and middle pulmonary vein draining to the superior vena cava (white arrows).
Figure 2.
Figure 2.
Chest radiograph.
Figure 3.
Figure 3.
Transthoracic echocardiography showing a) right heart dilatation in the apical four-chamber view and b) the sinus venosus atrial septal defect in the subxiphoid paediatric view (white arrow) measuring 1.9 cm at its maximal diameter.
Figure 4.
Figure 4.
Three-dimensional trans-oesophageal echocardiography showing the sinus venosus atrial septal defect a) before and b) after surgical closure.
See this image and copyright information in PMC

References

    1. Gatzoulis MA, Freeman SC, Webb GD, et al. . Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med 1999; 340: 839–846. - PubMed
    1. Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30: 256–265. - PubMed

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