Sinus venosus atrial septal defect with severe mitral stenosis: A rare presentation
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0975-2870.157101
We present a case of 32-year-old female patient with rheumatic mitral stenosis with sinus venosus atrial septal defect (ASD) and anomalous drainage of the right superior pulmonary vein into right atrium. Keywords: Atrial septal defect patch closure, mitral valve replacement, rheumatic mitral stenosis, sinus venosus atrial septal defect
Lutembacher's syndrome (LS) refers to the rare combination of congenital atrial septal defect (ASD) usually ostium secundum and acquired mitral stenosis. [1] Its association with sinus venosus (superior vena cava type) ASD with partial anomalous pulmonary venous connection is rarely described in the literature. The hemodynamic effect of this combination makes clinical diagnosis difficult due to wide variation of clinical presentation. Diagnosis of sinus venosus ASD with mitral stenosis is usually made by clinical presentation and confirmed by two-dimensional (2D) echocardiography and transesophageal echocardiography. [2] The standard management of sinus venosus ASD with mitral stenosis is patch closure of ASD with left atrialization of superior pulmonary vein and mitral valve replacement or commissurotomy. We report a rare case of 32-year-old female patient with rheumatic mitral stenosis with sinus venosus ASD and anomalous drainage of the right superior pulmonary vein, treated successfully by pericardial patch closure with left atrialization of superior pulmonary vein and mitral valve replacement.
A 32-year-old female patient presented with chief complaints of dyspnea on exertion and palpitation since 5 years which increases since 6 months. She had a history suggestive of rheumatic fever in childhood, and she was on regular penicillin prophylaxis. There was no history suggestive of cyanosis, congestive heart failure and embolic episode in the past. On examination-There was a precordial bulge with cardiac enlargement, heaving left parasternal impulse and a systolic thrill at upper left sternal border. On auscultation, first heart sound was accentuated and second widely split and fixed with P2 slightly accentuated. Grade three ejection systolic murmurs was audible at second left intercostals space and a loud delayed diastolic murmur from left sternal border to apex without the presystolic accentuation. On abdominal examination, liver was palpable four cm below intercostal space with no ascites. Electrocardiography shows right ventricular hypertrophy with right axis deviation and strain pattern. X-ray chest shows considerable cardiomegaly with prominent main pulmonary artery, right atrial enlargement, ventricular enlargement, plethora but no buttressing [Figure 1].
Considering all these clinical findings with no left atrial enlargement she was considered to have ASD with mitral stenosis. 2D echocardiography was suggestive of rheumatic valvular heart disease with severe mitral stenosis (MVA 0.9) with thickened calcified mitral valve leaflet and severe tricuspid regurgitation, pulmonary hypertension of 70 mmHg and ASD of sinus venosus type. Patient was initially decongested, and then surgery was performed. Midline sternotomy was performed with a conventional cannulation including bicaval drainage and cardiopulmonary bypass with moderate hypothermia (30°) was established. Heart was arrested by cold antegrade serosanguinous cardioplegia solution. Right atrium was opened by an oblique atriotomy and findings of 2D echo were confirmed. There was evidence of hugely dilated right atrium with 3 cm sinus venosus ASD with severely calcified mitral valve leaflet and dilated tricuspid annulus with small left atrium (LA). The key anatomic criterion for the intraoperative diagnosis of sinus venosus defect is overriding of the mouth of the superior vena cava across the intact muscular border of the fossa ovalis. The interatrial communication is then formed within the mouth of the overriding vein and outside the confine of fossa ovalis [Figure 2] and [Figure 3].
Thickened fibroses mitral valve leaflets were excised, and 23 mm TTK Chitra mitral prosthetic valve was sutured to the mitral annulus with 2-0 prolene in a continuous fashion. De vega's annuloplasty was done for tricuspid valve. Autologous pericardial patch closure of ASD (sinus venosus) was done using 5-0 prolene in such a way to baffle the right superior pulmonary vein into LA. Right atrium was closed, and heart chambers were deaired and aortic cross clamp was removed. Heart started beating after giving shock. Heparin reversal was done with injection protamine. Cardiopulmonary bypass was discontinued. Patient tolerated procedure well and shifted to the recovery room. Postoperative echocardiography showed a good prosthetic profile, the absence of residual shunt and a decrease of pulmonary hypertension from 70 mmHg to 40 mmHg.
Lutembacher's syndrome is considered as the combination of a congenital ASD and acquired rheumatic mitral stenosis, each modifies the hemodynamic and clinical expression of other. As ostium secundum is most common of interatrial communication (80%), LS is most commonly associated with ostium secundum defect, association of sinus venosus ASD with mitral stenosis is very rare, as sinus venosus ASD includes only 2-3% of ASD. [3] Mitral stenosis increases the shunt flow across an ASD and ASD decreases the gradient across the stenotic valve, thus underestimating the severity of mitral stenosis. Mitral stenosis in a suspected case of ASD is very important since if it is missed, it may result in high left atrial and pulmonary venous pressure after surgical closure of ASD. The hemodynamic effects of LS are usually due to the relative interplay of the effects of the mitral stenosis and the ASD, and the direction of blood flow depends greatly on the compliance of the right and left ventricles. [4] Thus, pulmonary vascular resistance, compliance of the right ventricle, size of ASD and severity of MS are recognized factors which influence the natural history and hemodynamic features in patients with this syndrome. [5] Patients with LS will have a history of rheumatic fever in childhood with dyspnea. Sinus venosus ASD of superior vena cava type is clinically similar to an equivalent ostium secundum ASD with two exceptions, first-the atrial pacemaker is ectopic so P wave axis is leftward and inverted P waves appears in lead 2, 3, aVF. Second the right hilum on chest X-ray may show localized ampullary dilatation of distal SVC as it joins the right atrium. 2D echocardiography both transthoracic and transesophageal confirms the diagnosis of ASD with mitral stenosis. The basic principle of repair is redirection of the anomalous pulmonary venous connection through interatrial communication into LA. In contrast to operative repair of ostium secundum ASD, the surgical approach for sinus venosus ASD is more complex and carries the risk of stenosis of superior vena cava or pulmonary veins, residual shunting, and sinoatrial node dysfunction. In our present case report, 32 years female presented with a history of rheumatic fever in childhood with all clinical findings suggestive of left to right shunt at the atrial level with mitral stenosis. 2D echo support the clinical findings. Intraoperatively findings of 2D echo were confirmed. There was superior caval ASD, which is located in the posterosuperior atrial septum, cranial to limbus with anomalous pulmonary vein at superior atriocaval junction. In this case, we performed simple patch closure by autologous untreated pericardial patch, which directs the pulmonary vein blood flow across the ASD into LA. Mitral valve leaflets were severely calcified with severe subvalvular crowding. Mitral valve replacement was done using 2-0 prolene in a continuous fashion. Modified DeVega's tricuspid ring annuloplasty done. Patient did well postoperatively and extubated after 6 h. Postoperative 2D echo reveals normal functioning valve with no residual shunt across the ASD patch with no superior vena cava stenosis.
[Figure 1], [Figure 2], [Figure 3]
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