Lutembacher syndrome is a rare cardiac abnormality characterized by a combination of congenital atrial septal defect (ASD) and acquired rheumatic mitral stenosis (MS).
Here researchers report a case of 18-year-old male with Lutembacher syndrome successfully treated percutaneously with transcatheter Accura balloon valvuloplasty and Amplatzer septal occluder device closure.
A 18-year-old male presented to hospital with 6 month history of dyspnea on exertion and palpitations. Patient gave history suggestive of rheumatic heart disease, diagnosed at peripheral hospital three years back and was on penicillin prophylaxis.
On auscultation loud first heart sound, fixed splitting of second heart sound, grade 3/6 ejection systolic murmur at pulmonary area and middiastolic murmur at apex was heard. His ECG showed sinus rhythm, tall P waves, right ventricular hypertrophy and incomplete right bundle branch block. X-ray chest showed cardiomegaly with pulmonary plethora. TTE revealed dilated right atrium and right ventricle. There was rheumatic mitral stenosis, a large ostium secundum ASD with left to right shunt and adequate rims. In TEE it was found to be a 30 mm ASD with superior vena cava rim of size 12 mm and inferior vena cava rim of size 7 mm and no left atrial or left atrial appendage clot.
Diagnosis of Lutembacher syndrome was made and the patient was stabilized with diuretics. Percutaneous trans-luminal mitral commissurotomy (PTMC) was done using 28 mm Accura balloon catheter introduced through ASD. Post procedure transesophageal and transthoracic echocardiogram showed ASD device in situ with no residual shunt. The patient was then discharged after two days in stable condition.
Indications for percutaneous transcatheter treatment of Lutembacher syndrome include:
(1) ASD with Qp/Qs ratio greater than 1.5 with adequate rims,
(2) symptomatic moderate to severe MS with valve morphology favorable for PTMC,
(3) any degree of pulmonary hypertension, except in patients with Eisenmenger syndrome (irreversible pulmonary hypertension).
Source: JAPI, India