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Biomedical subjects

J K Perloff

Publications and source records attributed to J K Perloff.

At least 19 recordsLinked to original sources
PubMed1990

Pregnancy and congenital heart disease.

Congenital heart disease as a complicating factor in pregnancy has assumed increasing clinical importance because improved techniques of surgical repair have resulted in a larger proportion of affected women living to the reproductive age. The most serious forms are those associated with pulmonary hypertension (such as the Eisenmenger syndrome), which carry a prohibitively high risk of maternal death. Complex forms of cyanotic heart disease, of which the commonest is the tetralogy of Fallot, are only slightly less dangerous. It has recently been recognized that children born to women with congenital heart disease are at increased risk of having cardiac defects; fetal echocardiography is therefore an important diagnostic test. Optimal care of the pregnant woman with congenital heart disease is best provided by a team consisting of internist-cardiologist, obstetrician-perinatologist, obstetric anesthesiologist, and ultrasonographer-echocardiographer.

Delivery, Obstetric↗
PubMed1990

Facioscapulohumeral muscular dystrophy: evidence for selective, genetic electrophysiologic cardiac involvement.

Facioscapulohumeral muscular dystrophy is an autosomal dominant disorder with an incidence of 3 to 10 cases per million. The only type of cardiac involvement ascribed to this neuromuscular disorder is a unique form of heart disease--permanent atrial paralysis. However, reported cases of facioscapulohumeral muscular dystrophy probably represented instead what is now recognized as phenotypically similar Emery-Dreifuss dystrophy. Cardiac involvement, therefore, has not been convincingly reported in facioscapulohumeral muscular dystrophy, but because of the clinical similarity of that disorder to Emery-Dreifuss dystrophy and its genetic variants, a prospective investigation of the electrophysiologic properties of the atria and atrioventricular (AV) node and infranodal conduction was undertaken in 30 rigorously documented cases of facioscapulohumeral muscular dystrophy. All patients had a 12 lead surface electrocardiogram (ECG), 22 had a 24 h ambulatory ECG, 15 patients had two-dimensional echocardiographic/Doppler studies and 10 patients underwent 12 intracardiac electrophysiologic investigations. Left atrial, right atrial or biatrial P wave abnormalities were present in 60% of the surface ECGs. Evidence of abnormal AV node or infranodal conduction was present on intracardiac electrophysiologic study or surface ECG in 27% of patients. Atrial flutter or fibrillation was induced by single atrial extra stimuli in 10 of the 12 intracardiac electrophysiologic studies. Sinus node function was abnormal in three patients. This investigation provides the first secure evidence of cardiac involvement in facioscapulohumeral muscular dystrophy. The involvement is represented by relatively high susceptibility to induced atrial flutter or fibrillation during electrophysiologic study, together with less frequent evidence of abnormal sinus node function and abnormal AV node or infranodal conduction.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Isolated noncompaction of left ventricular myocardium. A study of eight cases.

Isolated noncompaction of left ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses. This study comprised eight cases, including three at necropsy. Ages ranged from 11 months to 22.5 years, with follow-up as long as 5 years. Gross morphological severity ranged from moderately abnormal ventricular trabeculations to profoundly abnormal, loosely compacted trabeculations. Echocardiographic images were diagnostic and corresponded to the morphological appearances at necropsy. The depths of the intertrabecular recesses were assessed by a quantitative echocardiographic X-to-Y ratio and were significantly greater than in normal control subjects (p less than 0.001). Clinical manifestations of the disorder included depressed left ventricular systolic function in five patients, ventricular arrhythmias in five, systemic embolization in three, distinctive facial dysmorphism in three, and familial recurrence in four patients. We conclude that isolated noncompaction of left ventricular myocardium is a rare if not unique disorder with characteristic morphological features that can be identified by two-dimensional echocardiography. The incidence of cardiovascular complications is high. The disorder may be associated with facial dysmorphism and familial recurrence.

Abnormalities, Multiple↗
PubMed1979

Discrete fibromembranous aortic stenosis in middle age. Natural history and case report.

Discrete fibromembranous subaortic stenosis may be severe at birth, progress in severity irrespective of the initial degree of obstruction, and morphologically change during the course of its natural history. Initial status, progression of the lesion and perhaps later development of fibromuscular (tunnel) and/or asymmetric septal hypertrophy may all contribute to a shortened life span and account for the different incidence of fibromembranous subaortic stenosis in children and adults. In the case reported here, a patient survived into the sixth decade with a discrete fibromembranous subaortic stenosis, which led to typical as well as unusual echocardiographic findings.

Age Factors↗
PubMed1979

Left axis deviation: a reassessment.

This report deals with the ramifications of the concept of left axis deviation. In early life, the leftward shift of the frontal plane QRS axis is determined chiefly, if not solely, by the relative weights of the ventricles. Once adult ventricular weight ratios are reached, there is a long period of axis stability, then a gradual leftward drift of the QRS, governed principally by left anterior fascicular conduction. Thus, the normal QRS axis is age-dependent, and left axis deviation must be considered accordingly.

Adult↗
PubMed1977

Ventricular inversion without transposition of the great arteries. A rarity found in association with atresia of the left-sided (tricuspid) atrioventricular valve.

Clinical and morphologic features are described in a 13 week old male infant with ventricular inversion without associated transposition of the great arteries but with concomitant atresia of the left-sided (tricuspid) atrioventricular valve. Isolated ventricular inversion without situs inversus or visceral heterotaxy has previously been reported in English in only three patients, none with tricuspid atresia.

Abnormalities, Multiple↗
PubMed1977

Acute severe aortic regurgitation. Pathophysiology, clinical recognition, and management.

Acute severe aortic regurgitation is a relatively unfamiliar, though life-threatening, disease. We review its diverse causes, anatomic faults, and hemodynamic sequelae and set the stage for an understanding of the clinical manifestations in light of their physiologic mechanisms. Clinical information includes the natural history, physical signs (physical appearance, systemic arterial pulse, jugular venous pulse, precordial palpation, auscultation), electrocardiogram, and chest roentgenogram. Echocardiographic features are especially emphasized and the need for prompt diagnosis and surgical intervention underscored, even in the setting of active infective endocarditis.

Acute Disease↗