Mitral Stenosis, Sinus Venosus Atrial Septal Defect, and Partial Anomalous Pulmonary Venous Return

Mitral Stenosis, Sinus Venosus Atrial Septa1 Defect, and Partial Anomalous Pulmonary Venous Return: Diagnosis by Multiplane Transesophageal Echocardiography JAMSHID SHIRANI, M.D., DANNY WOO, M.D., WILLIAM GOTSIS, M.D., JAY S. MEISNER, M.D., and JOEL A. STROM, M.D. The Department of Medicine, Division of Cardiology, The Albert Einstein College of Medicine, Bronx, New York We describe a 42-year-old man with rheumatic mitral stenosis, sinus uenosus atrial septal defect, and anomalous drainage of the right upper pulmonary vein to the superior uena caua. Transthoracic echocardiography (TTE) failed to identify the atrial septal defect and the partial anomalous pulmonary venous return. Transesophageal echocardiography (TEE), using a multiplane probe, was useful in delineating the abnormalities. To our knowledge, this is the first reported patient with rheumatic mitral stenosis and sinus uenosus defect. (ECHOCARDIOGRAPm, Volume 13, November 1996) mitral stenosis, transesophageal echocardiography, sinus venosus atrial septal defect, partial anomalous pulmonary uenous return

The association between acquired mitral stenosis and left-to-right shunt at the atrial level has been known since its first comprehensive description by Lutembacher.' In the majority of patients reported since, the leftto-right shunt was the result of a patent foramen ovale or a secundum atrial septal defect.2 In a small minority, a primum atrial septal defect3 or a partially anomalous pulmonary venous return with intact atrial septum* may have been responsible for the shunt. We present a patient in whom a sinus venous atrial septal defect, an anomalous connection of the right upper pulmonary vein t o the superior vena cava and rheumatic mitral stenosis, was diagnosed by multiplane transesophageal echocardiography (TEE) and confirmed at surgery. A 42-year-old man presented to the Cardiology Clinic with complaints of palpitation, exertional dyspnea, and easy fatigability for 6

Address for correspondence and reprints: Jamshid Shirani, M.D., 1300 Morns Park Avenue, Room G-42, Bronx, NY 10461. Fax: 718-823-0032.

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months. On examination the neck veins were distended to the angle of the jaw, with prominent V waves. The pulse was irregular at a mean of 112 beatdmin and the blood pressure was 114/74 rnmHg. Precordial palpation revealed right ventricular (RV) heave and laterally displaced apical impulse. The first heart sound and the pulmonic component of the second heart sound were accentuated. A grade IINI diastolic rumble could be heard in the vicinity of the left ventricular (LV) apex. A 12lead ECG showed atrial fibrillation, right-axis deviation, and RV hypertrophy. Cardiomegaly, dilated pulmonary arteries, and engorged peripheral pulmonary vessels were seen on the chest X ray. The patient was then referred for echocardiography. Transthoracic echocardiogram (TTE) showed normal LV size and systolic function, a markedly dilated RV, moderate left and marked right atrial enlargement, and a dilated pulmonary trunk. The mitral valve was thickened with restricted mobility. The mitral valve area was 1.3 cm2by pressure halftime (patient in atrial flutter with variable AV block) and 0.6 cm2 by direct planimetry (Fig. 1).Mild tricuspid insufficiency and severe

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Figure 1. Transthoracic echocardiographic images of the heart. (A) Parasternal short-axis view of the mitral valve at the tip of the leaflets showing markedly thickened leaflets with restricted opening. The value area was 0.6 cm2 by planimetry. (B) Continuous-wave Doppler flow spectral of the mitral valve showing relatively preserved deceleration slope. Patient is in atrial flutter with variable atrioventricular block during this study. Pressure halftime was 170 msec.

pulmonary hypertension (estimated RV systolic pressure = 62 mmHg) were also present. No abnormalities could be seen in the atrial septum. However, because of the discrepancy between the mitral valve area by pressure halftime and planimetry, presence of marked right atrial and ventricular dilation, and enlargement of the pulmonary trunk, an atrial septal defect was suspected. TEE was performed using a 5-MHz multiplane probe (Hewlett-Packard, Andover, MA, USA). A sinus venosus atrial septal defect was found (2 cm in diameter). The defect was best visualized at a plane angulation of 104" (Fig. 2). The right upper pulmonary vein drained directly into the distal portion of the superior vena cava (Fig. 2). All other pulmonary veins drained normally into the left atrium (LA). Coronary angiography showed normal epicardial coronary arteries. Pulmonary-to-systemic shunt ratio was 4.6: 1.The patient underwent successful repair of the atrial septal defect and anomalously draining of the pulmonary vein and replacement of the mitral valve. The presence of atrial septal defect in a patient with mitral stenosis provides an alternate route for decompression of the LA. Consequently, LA pressure falls rapidly during diastole, resulting in a shortened pressure halftime and overestimation of mitral valve

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area.5 Measurement of the mitral valve area by direct planimetry, however, remains accurate in this ~ondition.~ In our patient, estimation of the mitral valve area by pressure halftime was also complicated by the presence of atrial flutter.'j Sinus venosus atrial septal defect is uncommon in the adult and is not reliably detected by TTE because the superior portion of the atrial septum is not well imaged.7 In addition, presence of the commonly associated anomalous drainage of the right upper pulmonary vein can not be assessed by the transthoracic approach. TEE,7 especially using a multiplane probe,s allows complete visualization of the atrial septum and assessment of pulmonary venous drainage, and is thus, as shown in our patient, the best available noninvasive method of evaluating patients with mitral stenosis and atrial septal defect. To our knowledge, noninvasive diagnosis of mitral stenosis, sinus venosus atrial septal defect, and partial anomalous pulmonary venous return using TEE has not been previously reported.

References 1. Lutembacher R: De la stenose mitrale avec communication interauriculaire. Arch Ma1 Coeur 1916;9:237-244.

ECHOCARDIOGRAPHY: A Jrnl. of CV Ultrasound & Allied Tech.

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NIITRAL STENOSIS AND SINUS VENOSUS DEFECT

Figure 2. Multiplane transesophageal echocardiographic images of the heart. (A) Basal four-chamber view showing thickened mitral valve (Mv),and dilated right atrium (RA)and right ventricle (RV). (B) View of the atrial septum showing the sinus venosus defect (arrowhead). The right upper pulmonary vein (RUPV) drains into the distal portion of the superior uena caua (SVC). (C) Color flow Doppler and (0) contrast (agitated saline) further delineate the sinus venous defect (arrowhead). L A = left atrium; RA = right atrium; RAA = right atrial appendage. Bashi W, Ravikumar E, Jairaj PS, et al: Coexistent mitral valve disease with left-to-right shunt at the atrial level: Clinical profile, hemodynamics, and surgical considerations in 67 consecutive patients. Am Heart J 1987;114: 1406-1414. Wassermil M, Hoffman MS: Partial anomalous pulmonary venous drainage associated with mitral stenosis with an intact atrial septum. Am J Cardiol 1962;10:894-899. Shah MK, Bhat A, Venkitachalam CG: Ostium primum atrial septal defect with rheumatic mitral stenosis. Zndian Heart J 1992;44:189-191. Vasan RS, Shrivastava S, Kumar M V : Value and limitations of Doppler echocardiographic

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determination of mitral valve area in Lutembacher syndrome. JACC 1992;20:1362-1370. 6. Hatle L, Angelson B, Tromsdal A: Noninvasive assessment of atrioventricular pressure halftime by Doppler ultrasound. Circulation 1979; 60:1096-1104. 7. Kronzon I, Tunick PA, Freedberg RS, et al: Transesophageal echocardiography in superior to transthoracic echocardiography in the diagnosis of sinus venosus atrial septal defect. JACC 1991;17:537-542. 8. Maxted W, Sanyal R: Multiplane transesophageal echocardiographic detection of sinus venosus atrial septal defect. Echocardiography 1995;12:139-143.

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