Case Report

A 21 - year- old male was admitted to our hospital with dyspnea. Transthoracic echocardiography and cardiac catheterization revealed a infundibular pulmonary stenosis with a pressure gradient of 80 mm Hg. Cardiac catheterization pointed out no additional pathology(Figure 1).

Figure 1

Figure 1

He was operated under endotracheal general anesthesia and in supine position. Perioperatively, it was revealed that infundibular region and pulmonary valve were completely normal; whereas inflow region of the right ventricle showed stenosis. (Figures 2 and 3).

Figure 2

Figure 2

Figure 3

Figure 3

Hypertrophied muscle bundles at the stenotic region of the pulmonary inflow were resected (Figure 4).

Figure 4

Figure 4

21 mm Hegar dilator could be passed easily through resected area which indicated that adequate dilation was obtained (Figure 5).

Figure 5

Figure 5

Incisions of right ventriculotomy and pulmonary artery were closed primarily(Figure 6).

Figure 6

Figure 6

The post-operative course was uneventful. Histologic examination revealed cardiac muscle cell hypertrophy and disorganization.Postoperative echocardiographic data confirmed complete correction of the lesion.

Comments

Double-chambered right ventricle is a rare congenital heart disease(₁). The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis(₂).

Accurate determination of the severity of the stenosis and the anatomy of the obstructing lesion are important in devising a treatment strategy(₃). Multiplane transesophageal echocardiography provides excellent views of an anomalous muscle bundle in the right ventricle, which differed from a moderator band by its insertion site on the right ventricle(₁).

Radical correction for two-chambered right ventricle in Shimizu et al study was classified into 3 groups according to the method used for releasing the stenosis in the right ventricle; Group A: resection of thickened fibrous tissue; Group B: resection of the fibrous tissue and abnormal muscular bundles; and Group C: resection of the fibrous tissue and abnormal muscular bundles with patch-enlargement of the right ventricular outflow tract(₄).Generally, resection of the hypertrophic muscle bundles practically eliminated the obstruction(₅).

Correspondence to

Doç. Dr. Ufuk YETKIN, 1379 Sok. No: 9,Burç Apt. D: 13 - 35220, Alsancak - IZMIR / TURKEY Tel: +90 505 3124906 , Fax: +90 232 2434848 e-mail: ufuk_yetkin@yahoo.fr