J Cardiovasc Ultrasound.  2009 Mar;17(1):25-27. 10.4250/jcu.2009.17.1.25.

Inferior Sinus Venosus Type Atrial Septal Defect Initially Presenting Pulmonary Hypertension on Transthoracic Echocardiography

• Cho HJ
• Chung WJ
• Bong JM
• Shin KC
• Shin MS
• Kang WC
• Han SH
• Moon CI
• Koh KK
• Ahn TH
• Choi IS
• Shin EK

• KMID: 1473767
• DOI: http://doi.org/10.4250/jcu.2009.17.1.25

Abstract

Inferior sinus venosus type atrial septal defect (ASD) is a rare congenital cardiac deformity that occurs between the inferior vena cava and right atrium. Inferior sinus venosus defect is difficult to diagnose through transthoracic echocardiography because of its location which is infero-posterior to the fossa ovalis. Increasing pulmonary arterial pressure and pulmonary vascular resistance in patients with sinus venosus defect usually occur earlier than other types of ASD. We report a case of 19-year-old man who presented exertional dyspnea due to inferior sinus venous type ASD with mild pulmonary hypertension. In this case, we found clues from slight diastolic flattening of interventricular septum and shortened acceleration time of right ventricular outflow tract on initial transthoracic echocardiography, leading right heart catheterization and transesophageal echocardiography to reveal this rare type of ASD.

Keyword

Echocardiography; Atrial septal defect; Pulmonary hypertension

MeSH Terms

Acceleration
Arterial Pressure
Cardiac Catheterization
Cardiac Catheters
Congenital Abnormalities
Dyspnea
Echocardiography
Echocardiography, Transesophageal
Heart Atria
Heart Septal Defects, Atrial
Humans
Hypertension, Pulmonary
Vascular Resistance
Vena Cava, Inferior
Young Adult

Figure

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  Fig. 1 2D views of transthoracic echocardiography. A: Parasternal short axis view at mid-LV level showed slight diastolic flattening of interventricular septum (arrows). B: Apical 4 chamber view showed mildly dilated right ventricle and atrium. LV: left ventricle.

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  Fig. 2 Doppler findings. A: PAsP from TRV was within normal range. B: RVOT AT was 88.7 msec and calculated mPAP from the simplified Mahan's equation (mPAP= 80-[RVOT AT×0.5]) was 35.6 mmHg (B). PAsP: pulmonary arterial systolic pressure, TRV: tricuspid regurgitation velocity, RVOT: right ventricular outflow tract, AT: acceleration time, mPAP: mean pulmonary arterial pressure.

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  Fig. 3 Transesophageal echocardiography. Color Doppler imaging showed a defect at the extremely posterior portion of interatrial septum with left to right shunt flow.

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  Fig. 4 Patch repair of inferior sinus venosus type atrial septal defect (ASD) with autopericardium. The defect was located in inferior vena cava area and size was 1.5 cm. CS: coronary sinus, IVC: inferior vena cava.

Reference

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