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Lutembacher's syndrome: a case report.

INTRODUCTION: Lutembacher's syndrome refers to a congenital atrial septal defect (ASD) complicated by acquired mitral stenosis (MS). (1) It comprises of Atrial Septal Defect (ASD) [Ostium Secundum] with Mitral stenosis (MS). Inter-atrial septum develops from two sources-septum primum and septum secundum. If defect is in the formation of septum primum-it leads to the formation of ASD (Primum) and if defect is in the formation of septum secundum it forms ASD (secundum). Here, we will discuss about a lady who was diagnosed as having Lutembacher's syndrome at the age of 40 years.

CASE REPORT: A 40 year old female presented with chief complaints of palpitation since one month and easy fatigability for 20 days. Palpitation was paroxysmal in onset and usually occurred during more than daily routine activity and subsided by taking rest. There was no history of accompanying chest pain, cough, hemoptysis, fever or symptoms of upper respiratory tract infections. There was no past history of fever, joint pains with throat infection, rashes or abnormal body movements, or any long term medications for any premorbid condition. She was married since 20 years, had three children and all three deliveries were uneventful.

On general examination, she was afebrile with hypovolemic regular pulse of 84 beats/min, normal jugular venous pressure, no clubbing, no cyanosis and no pedal edema. Blood pressure was 114/80 mm of Hg, with respiratory rate of 16 breaths/min.

On cardiac examination, diffuse apical impulse was found in 6th ICS just lateral to midclavicular line and there was grade-1 left parasternal heave. On auscultation there was a loud S1 and a mid-diastolic murmur at mitral area, pansystolic murmur was heard at tricuspid area, an ejection systolic murmur in pulmonary area (flow murmur) with wide and fixed split S2.

Respiratory system examination revealed bilaterally equal normal vesicular breath sounds and no added sounds. Abdominal examination revealed no organomegaly.

Her investigation reports were as follows:

* Hemoglobin (Hb)--11.4 gm.%.

* White blood cells (WBC)--7800/[mm.sup.3]

* P= 64%, L= 30%, M= 04%, E= 02%, B= 00 %.

* Platelet count--1.28 lakh;

* Random blood sugar--94 mg/dl;

* Blood urea--29 mg/dl;

* Serum creatinine--0.7 mg/dl;

* Prothrombin time (test) = 13.5 sec. (Range= 10-14 sec.).

* Prothrombin time (control) = 12.2 sec.

* INR = 1.08

* aPTT = 24.2 sec. (Range = 30-40 sec.).

* Serum total cholesterol--186.6 mg%.

* Serum triglyceride--109.5 mg%.

* Serum LDL--123.5 mg%.

* Serum VLDL- 21.9 mg%.

* Serum HDL- 41.2 mg%.

* Urine Routine and Microscopic examination was Normal.

Electrocardiogram [Figure 1] [right arrow] Normal Sinus Rhythm; Biatrial enlargement; Right axis deviation (115), Normal PR Interval/Normal QTc.

[FIGURE 1 OMITTED]

Chest x-ray posteroanterior view [Figure 2] shows prominence of left pulmonary artery, cardiomegaly predominantly showing right ventricular enlargement and straightening of left border of heart.

[FIGURE 2 OMITTED]

2D-Echocardiography [Figures 3 & 4] [right arrow] severe mitral stenosis; mitral valve area 0.80 [cm.sup.2]. Large Ostium Secondum atrial septal defect (3.6-4.0 cm) with Left. To Right Shunt, mild MR, moderate to severe TR, moderate PAH. No vegetation, no clot, no thrombus and intact IVS.

[FIGURE 3 OMITTED]

Figure 3: Transthoracic echocardiogram of patient of Lutembacher's syndrome showing thickened mitral leaflets.

[FIGURE 4 OMITTED]

Figure 4: Transthoracic echocardiogram color Doppler of patient of Lutembacher's syndrome showing flow from left atrium to right atrium through atrial septal defect.

[FIGURE 5 OMITTED]

Figure 5: Transthoracic echocardiogram color Doppler of patient of Lutembacher's syndrome showing moderate to severe tricuspid regurgitation.

[FIGURE 6 OMITTED]

Figure 6: Transthoracic echocardiogram color Doppler of patient of Lutembacher's syndrome showing tricuspid regurgitation velocity.

[FIGURE 7 OMITTED]

Figure 7: Transthoracic echocardiogram color Doppler of patient of Lutembacher's syndrome showing pulmonary regurgitation.

DISCUSSION: In Lutembacher's syndrome, initially, high left atrial pressure due to mitral stenosis is thought to stretch open the patent foramen ovale, causing left-to-right shunt and providing another outlet for the left atrium. The hemodynamic effects of this syndrome are a result of the interplay between the relative effects of ASD and mitral stenosis. In our case there was a large ASD, so it masked the signs and symptoms of mitral stenosis. Development of Eisenmenger syndrome or irreversible pulmonary vascular disease is very uncommon in the presence of large ASD and high left atrial pressure because of mitral stenosis.

The incidence of this condition is very rare. In one study published in American Heart Journal in 1997, it was found that the incidence of Lutembacher's syndrome was- 0.001/10, 00000. (2) The ameliorating role of the ASD in MS was evident in Lutembacher's original report of 1916; the patient was a 61-year-old woman who had been pregnant seven times. (3) An earlier case report in the literature in 1880 (and referred to by Perloff). (1) was of a 74-year-old woman who had endured 11 pregnancies.

Early diagnosis and surgical treatment bears a good prognostic value. If patient is diagnosed at late stage, pulmonary hypertension and heart failure develops and the prognosis is bad. (4) If the patient is diagnosed earlier before the development of pulmonary hypertension and heart failure, ASD closure with mitral valve replacement bears a good prognosis and prolongs survival.

DOI: 10.14260/jemds/2014/2208

REFERENCES:

(1.) Perloff JK. The clinical recognition of congenital heart disease. 4th ed. Philadelphia: Saunders; 1994. p. 323-8.

(2.) Ali SY, Rahman M, Islam M, Barman RC, Ali MY, Islam MMSU et al. Lutembacher's Syndrome- A Case Report. Faridpur Med Coll J 2011; 6:59-60.

(3.) Lutembacher R. De la stenose mitrale avec communication interauriculaire. Arch Mal Coeur 1916; 9:237-60.

(4.) Levin AR, Spach MS, Boimeau JP, Canent RV Jr, Capp MP, Jewett PH. Atrial Pressure flow dynamics and atrial septal defects (Secundum type). Circulation 1968; 37:476-88.

Hari Om Gupta [1], Shailendra Manjhwar [2], Devendra Jain [3], Saurabh Gupta [4]

AUTHORS:

[1.] Hari Om Gupta

[2.] Shailendra Manjhwar

[3.] Devendra jain

[4.] Saurabh Gupta

PARTICULARS OF CONTRIBUTORS:

[1.] Assistant Professor, Department of General Medicine, Shyam Shah Medical College, Sanjay Gandhi Memorial Hospital.

[2.] Assistant Professor, Department of General Medicine, Shyam Shah Medical College, Sanjay Gandhi Memorial Hospital.

[3.] Post Graduate Student, Department of General Medicine, Shyam Shah Medical College, Sanjay Gandhi Memorial Hospital.

[4.] Post Graduate Student, Department of General Medicine, Shyam Shah Medical College, Sanjay Gandhi Memorial Hospital.

NAME ADDRESS EMAIL ID OF THE CORRESPONDING AUTHOR:

Dr. Hari Om Gupta, F-2, Doctor's Colony, Medical College Campus, Rewa, M. P. E-mail: guptadr.hariom@yahoo.co.in

Date of Submission: 16/02/2014.

Date of Peer Review: 17/02/2014.

Date of Acceptance: 24/02/2014.

Date of Publishing: 12/03/2014.
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Article Details
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Author:Gupta, Hari Om; Manjhwar, Shailendra; Jain, Devendra; Gupta, Saurabh
Publication:Journal of Evolution of Medical and Dental Sciences
Article Type:Case overview
Date:Mar 17, 2014
Words:1090
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