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Biomedical subjects

I Nazarian

Publications and source records attributed to I Nazarian.

15 recordsLinked to original sources

Right ventricular endomyocardial fibrosis. Diagnosis and management.

The clinical course, noninvasive and invasive diagnostic findings in 4 patients (pt) with endomyocardial fibrosis are reported. All patients (16-50 years) were in functional class III-IV (NYHA). Central venous pressure with large a- and v-waves was elevated in all; liver enlargement and peripheral edema were also noticed in all pts, and ascites in 3. An apical 2/6 systolic murmur was present in 2, and a right parasternal pansystolic murmur 2-4/6 with positive Carvallo's sign in all pts. ECG was non-specific and chest x ray showed right atrial enlargement in every case. A right-sided diastolic plateau which was higher than left ventricular end-diastolic pressure was present in all pts. Echo-, angiocardiography and computed tomography of the heart revealed obliteration of the right ventricular cavity, predominantly localized at the apex. Two pts who underwent endocardial resection and tricuspid valve replacement are alive and well after 9 and 8 years, respectively. One pt died early in the postoperative period and one died waiting for surgical therapy. In conclusion, echo-, angiocardiography and computed tomographic findings are diagnostic. A satisfactory differentiation from other cardiac disorders with restrictive hemodynamics and right-sided heart failure is possible.

Adolescent

Constrictive endocarditis. Report of a case with successful surgery.

An 18-year old white youth presented with severe right heart failure and was found to have an obliterated and funnel-shaped right ventricle, massive tricuspid regurgitation, and mitral regurgitation. The haemodynamic findings were those of cardiomyopathy with obliteration on the right side. He underwent surgery consisting of decortication and peeling off of a thickened right ventricular endocardium, tricuspid valve replacement, and mitral valve repair. After surgery there was pronounced haemodynamic as well as clinical improvement. The pathological picture was that of constrictive endocarditis. We recommend this method of treatment for patients with obliterate cardiomyopathy on either side of the heart.

Adolescent

Quantitative angiocardiographic recognition of atypical form of tetralogy of Fallot with absent pulmonic valve.

A 9-year-old boy is reported who had tetralogy of Fallot, rudimentary pulmonic valve, and supravalvar pulmonic stenosis. The patient lacked almost all of the clinical findings commonly encountered in this syndrome. Thus he never had signs of a large left-to-right shunt, or congestive heart failure. He had no murmur of pulmonic regurgitation and his pulmonary artery was not large on chest roentgenogram. Angiocardiography revealed moderate main pulmonary arterial dilation. The lack of the usual manifestations of tetralogy of Fallot with absent pulmonic valve was due to supravalvar pulmonic stenosis, acting as a natural pulmonary artery band. A quantitative angiocardiographic study was undertaken in order to find a clue for the diagnosis of these atypical cases. The ratio of the transverse diameters of the main pulmonary artery and the aortic root as measured on lateral angiocardiograms in 31 children with uncomplicated tetralogy of Fallot was 0.70 +/- 0.22, whereas this ratio was 1.70 in this particular patient (p less than 0.001). It is concluded that a quantitative evaluation of the ratio of the transverse diameter of the main pulmonary artery to the aortic root on lateral angiocardiogram allows differentiation of tetralogy of Fallot with absent pulmonic valve associated with supravalvular pulmonic stenosis from uncomplicated forms of tetralogy of Fallot.

Angiocardiography

Association of atrioventricular valve atresia with single ventricle, truncus arteriosus communis and transposition. A basic reorientation in the approach to the definition of congenital heart defects.

A heart specimen is presented which showed a rare combination of atrioventricular valve atresia with single ventricle, truncus arteriosus communis with an anteriorly placed trunk, ie transposition. Because of the problems involved in the classification of this heart, the types of single ventricle are revised with particular attention to the rare cases of atrioventricular valve atresia and single ventricle reported in the literature. Also the rare type of truncus arteriosus communis with truncoatrioventricular valve discontinuity is excluded from the so-called transposition complexes, and properly classified as a form of truncus. The literal meaning of transposition is stressed and it is urged to approach congenital heart defects in a purely anatomic sense with definition of each segment as accurately as possible. Thus the present confusion in terminology and further plethora of new terms are avoided.

Child

Isolated unilateral absence of the pulmonary artery. Review of the world literature and guidelines for surgical repair.

A 10-month-old boy is presented who had isolated unilateral absence of the right pulmonary artery. He suffered from hemoptysis and severe congestive heart failure. The patient underwent prosthetic anastomosis of the right to the main pulmonary artery. Although the hemodynamic response was favorable, his oxygenation did not improve due to diffuse pulmonary arteriovenous fistulae of the affected lung. The patient succumbed 3 months after operation due to massive uncontrollable hemoptysis from the right lung. Isolated unilateral absence of the pulmonary artery is a rare lesion. In our review of the world literature as of November 1976, 47 cases (including this report) of the unilateral absence of the pulmonary artery have been reported. Of these 25.5% had pulmonary hypertension and only 4 cases underwent successful repair of the lesion. Though repair of this defect can be carried out, the result may not be always gratifying. Our experience with this case has led us to consider a lung biopsy before proceeding to the surgical repair of the lesion. If the affected lung shows arteriovenous abnormalities the operation should not be recommended.

Blood Vessel Prosthesis

Calcified right ventricular fibroma causing outflow obstruction. Report of a case with successful excision.

A 5-year-old boy with clinical findings of pulmonic stenosis was found to have a large calcified mass in the right ventricular outflow region and a gradient of 120 mm Hg between the right ventricle and the pulmonary artery. At surgery, an ovalshaped tumor attached to the interventricular septum and obstructing the right ventricular outflow tract was removed. The child survived and is doing well. Histologically, the tumor had the characteristics of fibroma. A hemodynamic study three months after surgery showed almost complete abolishment of the gradient. To our knowledge this is the fifth reported case of calcified right ventricular fibroma with successful operation. In childhood intracardiac calcifications, together with obstruction, are highly suggestive of this lesion.

Angiocardiography