Search PubMed⌕ Search

Biomedical subjects

J K Perloff

Publications and source records attributed to J K Perloff.

At least 37 records · Page 2Linked to original sources
PubMed1994

Congenital heart disease and pregnancy.

In Europe and North America, a dramatic fall in the incidence of rheumatic fever and rheumatic heart disease has coincided with advances in medical and surgical management of congenital heart disease and has resulted in a shift in the relative incidence of these two categories of cardiac disorders in women of child-bearing age. This review deals with pregnancy and congenital heart disease--unoperated and operated. Central to this topic is the intricate interplay between maternal circulatory and respiratory physiology and maternal congenital heart disease, and the effects of this interplay upon the fetus which is exposed to risks that threaten its intrauterine viability and to risks that subsequently express themselves as developmental defects or transmitted congenital malformations of the heart or circulation.

Delivery, Obstetric↗
PubMed1994

Congenital heart disease in the adult: clinical approach.

The remarkable advances in diagnostic techniques and in the surgical and medical care of infants and children with congenital heart disease have resulted in an impressive increase in longevity. Congenital heart disease in adults has emerged as a specialized area of cardiovascular interest. In response, special multidisciplinary facilities have been developed for the comprehensive care of adults with congenital heart disease. The scope of the discipline is relatively broad and includes unoperated survival patterns; survival patterns after surgery or interventional catheterization; a host of medical considerations; surgical considerations (operation and reoperation, cardiac catheterization as a therapeutic intervention, and noncardiac surgery); and the important problems of postoperative residua and sequelae. The purpose of this communication is to call attention to this subspecially and to excite the interest of radiologists in certain imaging aspects of congenital heart disease in adults.

Adult↗
PubMed1994

Pulmonary atresia with ventricular septal defect in adults.

BACKGROUND: Multistage surgery culminating in completed hemodynamic repair is now performed for pulmonary atresia with ventricular septal defect (PA-VSD). Justification for operation in patients with an adequate collateral pulmonary circulation is controversial. Data on natural adult survival are scant but are necessary to provide the rationale for multistage reconstructive procedures. METHODS AND RESULTS: All cyanotic adults with PA-VSD in the UCLA Adult Congenital Heart Disease Center Registry from 1978 through 1992 formed the basis for this study. Registry data and echocardiographic, hemodynamic, and angiographic information were used to determine longevity, clinical course, and operative feasibility. Of 26 patients, 16 were unoperated when referred (group A), and 10 had been palliated before age 18 years (group B). Two thirds were 18 to 29 years old. Only 2 patients survived beyond age 40 years. Six died during follow-up at a mean age of 31 years (+/- 12.1 SD). Eight group A patients were in New York Heart Association class II, and 8 were in class III. Of the 26 group A and B patients, 20 had aortic regurgitation, which was moderate or severe in 10. Eight had cardiac failure. Of 11 group A patients who remained unoperated, 5 died. Twelve patients were considered eligible for surgery at > or = 18 years of age. Ten underwent completed hemodynamic repair with a mean post-operative right ventricular-to-left ventricular systolic pressure ratio of 0.45 (+/- 0.16 SD), and there were no early or late deaths. CONCLUSIONS: Even when collateral blood flow permits adult survival, all such patients are symptomatic. Mean life expectancy without operation did not exceed three decades. Aortic regurgitation and cardiac failure are significant negative variables. Nearly half of unoperated adults died during follow-up. Staged hemodynamic repair can be performed with a low surgical risk in properly selected adults with PA/VSD.

Adult↗
PubMed1993

Risk of stroke in adults with cyanotic congenital heart disease.

BACKGROUND: Adults with cyanotic congenital heart disease and elevated hematocrit levels are often phlebotomized because of an assumed risk of cerebral arterial thrombotic stroke. Whether a relation exists between hematocrit level, symptomatic erythrocytosis (hyperviscosity), and stroke remains to be established in this patient population. METHODS AND RESULTS: Accordingly, 112 cyanotic patients 19-74 years old (mean, 36 +/- 11.7 years) in the UCLA Adult Congenital Heart Disease Center Registry were selected for study by virtue of continuous observation for 1-12 years (total, 748 patient-years). Patients with independent risk factors for embolic or vasospastic stroke were excluded. The study patients were then divided into two groups: 1) compensated erythrocytosis (stable hematocrit levels of 46.0-72.7% [mean, 57.5 +/- 7.2%], iron replete, absent or mild hyperviscosity symptoms), and 2) decompensated erythrocytosis (unstable rising hematocrit levels of 61.5-75.0% [mean, 69.5 +/- 10.6%], iron deficiency, marked-to-severe hyperviscosity symptoms). No patient with either compensated or decompensated erythrocytosis, irrespective of hematocrit level, iron stores, or the presence, degree, or recurrence of cerebral hyperviscosity symptoms, progressed to clinical evidence of a complete stroke (cerebral arterial thrombosis with brain infarction). CONCLUSIONS: Because a risk of stroke caused by cerebral arterial thrombosis was not demonstrated, because the circulatory effects of phlebotomy are transient, and because of the untoward sequelae of phlebotomy-induced iron deficiency, we recommend phlebotomy for the temporary relief of significant, intrusive hyperviscosity symptoms but not for the hematocrit level per se. According to our data, phlebotomy is not warranted to reduce an assumed risk of stroke because that risk did not materialize.

Adult↗
PubMed1993

Anesthetic implications of adults with congenital heart disease.

In adults with congenital heart disease who are confronted with noncardiac surgery, perioperative risks can be reduced, often appreciably, when problems inherent to this patient population are anticipated. The first necessity is to clarify the diagnosis and to be certain that appropriate information is obtained from a cardiologist with adequate knowledge of congenital heart disease in adults. Physiology and anatomy can vary significantly among patients who superficially carry identical diagnoses and would seem to fit under the same rubric. Elective noncardiac surgery should be preceded by clinical cardiovascular assessment, including reviewing clinical and laboratory data and securing necessary diagnostic studies. Preoperative assessment should be performed far enough in advance of the anticipated surgery to allow for critical assessment of the data. Appropriate cardiovascular laboratory studies to be obtained or reviewed include electrocardiograms, chest radiographs, echocardiograms, and cardiac catheterization data (which may include specialized intracardiac electrophysiologic testing). Congenital heart disease in adults is a new and evolving area of special interest and expertise in cardiovascular medicine. Multidisciplinary centers for the care of these patients are being developed. The 22nd Bethesda Conference recommended that these centers include anesthesiologists with special expertise in managing patients with congenital heart disease among their consultants. These anesthesiologists can function either as attending anesthesiologists when patients require noncardiac surgery, or as consultants and resource individuals to other anesthesiologists. Adults with congenital heart disease may present with age-related acquired cardiovascular and noncardiovascular disorders in addition to postoperative cardiac residua and sequelae, all of which require meticulous preoperative planning and consultation before noncardiac surgery is performed. We recommend that, particularly in larger departments of anesthesiology, select members of the department serve as consultants to anesthesiologists and to other members of the medical staff for these cases, especially when large numbers of adults with congenital heart disease are to be treated.

Adolescent↗
PubMed1993

Systemic complications of cyanosis in adults with congenital heart disease. Hematologic derangements, renal function, and urate metabolism.

The regulation of red cell mass, the hemostatic derangements, the structure and function of the kidneys and the determinants of plasma urate levels are important concerns in the clinical management of adults with cyanotic congenital heart disease. Because these topics are interrelated, they are discussed together in terms of pathogenesis, clinical expression, and management.

Arthritis, Gouty↗
PubMed1993

Transesophageal echocardiography in congenital heart disease in the adult.

Progress achieved during the last three decades in medical and surgical treatment of infants and children with congenital heart disease has resulted in a significant number of patients surviving to adulthood. The complexity of the basic malformations in addition to the superimposed surgical interventions makes accurate diagnostic imaging essential. The scope of information obtainable from high-quality TEE supports its use in the follow-up of operated and unoperated adult patients, as well as in surgical and interventional procedures. Application of the segmental analysis of anatomy is as important during TEE as it is during TTE. Supplemental transthoracic 2-D and spectral Doppler flow measurements may be needed when poor beam alignment during TEE precludes accurate gradient determinations. Although, to date, most reports of TEE in patients with congenital heart disease have involved children and adolescents, the informational advantage is potentially greater in older adolescents and adults in whom transthoracic windows are frequently limited. In our experience with 125 TEE studies in adult patients with congenital heart disease (the majority using a biplane probe), horizontal plane TEE imaging provided important additional information concerning pulmonary venous connections, atrial baffle function, atrioventricular anatomy and function, left ventricular outflow tract lesions, and great artery positional relationships. Vertical plane TEE enhances imaging of systemic and pulmonary venous connections, atrial situs, venosus atrial septal defects, atrioventricular anatomy and function, and the ventricular septum and outflow tracts and is essential for complete assessment of complex right ventricular outflow tract anatomy, ventriculoarterial alignment, and the sizing of all aortic segments in coarctation of the aorta.

Adult↗
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↗
PubMed1989

The limited reliability of physical signs for estimating hemodynamics in chronic heart failure.

The cardiovascular physical examination is used commonly as a basis for diagnosis and therapy in chronic heart failure, although the relationship between physical signs, increased ventricular filling pressure, and decreased cardiac output has not been established for this population. We prospectively compared physical signs with hemodynamic measurements in 50 patients with known chronic heart failure (ejection fraction, .18 +/- .06). Rales, edema, and elevated mean jugular venous pressure were absent in 18 of 43 patients with pulmonary capillary wedge pressures greater than or equal to 22 mm Hg, for which the combination of these signs had 58% sensitivity and 100% specificity. Proportional pulse pressure correlated well with cardiac index (r = .82), and when less than 25% pulse pressure had 91% sensitivity and 83% specificity for a cardiac index less than 2.2 L/min/m2. In chronic heart failure, reliance on physical signs for elevated ventricular filling pressure might result in inadequate therapy. Conversely, the adequacy of cardiac output is assessed reliably by pulse pressure. Our results facilitate decisions regarding treatment in chronic heart failure.

Adult↗