Reliability of multicenter pediatric echocardiographic measurements of left ventricular structure and function: the prospective P(2)C(2) HIV study.

BACKGROUND: To assess the reliability of pediatric echocardiographic measurements, we compared local measurements with those made at a central facility. METHODS AND RESULTS: The comparison was based on the first echocardiographic recording obtained on 735 children of HIV-infected mothers at 10 clinical sites focusing on measurements of left ventricular (LV) dimension, wall thicknesses, and fractional shortening. The recordings were measured locally and then remeasured at a central facility. The highest agreement expressed as an intraclass correlation coefficient (ICC=0.97) was noted for LV dimension, with much lower agreement for posterior wall thickness (ICC=0.65), fractional shortening (ICC=0.64), and septal wall thickness (ICC=0.50). The mean dimension was 0.03 cm smaller in central measurements (95% prediction interval [PI], -0.32 to 0.25 cm) for which 95% PI reflects the magnitude of differences between local and central measurements. Mean posterior wall thickness was 0.02 cm larger in central measurements (95% PI, -0.18 to 0.22 cm). Mean fractional shortening was 1% smaller in central measurements. However, the 95% PI was -10% to 8%, indicating that a fractional shortening of 32% measured centrally could be anywhere between 22% and 40% when measured locally. Central measurements of mean septal thickness were approximately 0.1 cm thicker than local ones (95% PI, -0.18 to 0.34 cm). Centrally measured wall thickness was more closely related to mortality and possibly was more valid than local measurements. CONCLUSIONS: Although LV dimension was reliably measured, local measurements of LV wall thickness and fractional shortening differed from central measurements.

Cardiac dysfunction and mortality in HIV-infected children: The Prospective P2C2 HIV Multicenter Study. Pediatric Pulmonary and Cardiac Complications of Vertically Transmitted HIV Infection (P2C2 HIV) Study Group.

BACKGROUND: Left ventricular (LV) dysfunction is common in children infected with the human immunodeficiency virus (HIV), but its clinical importance is unclear. Our objective was to determine whether abnormalities of LV structure and function independently predict all-cause mortality in HIV-infected children. METHODS AND RESULTS: Baseline echocardiograms were obtained on 193 children with vertically transmitted HIV infection (median age, 2.1 years). Children were followed up for a median of 5 years. Cox regression was used to identify measures of LV structure and function predictive of mortality after adjustment for other important demographic and baseline clinical risk factors. The time course of cardiac variables before mortality was also examined. The 5-year cumulative survival was 64%. Mortality was higher in children who, at baseline, had depressed LV fractional shortening (FS) or contractility; increased LV dimension, thickness, mass, or wall stress; or increased heart rate or blood pressure (P0.02 for each). Decreased LV FS (P<0.001) and increased wall thickness (P=0.004) were also predictive of increased mortality after adjustment for CD4 count (P<0.001), clinical center (P<0.001), and encephalopathy (P<0.001). FS showed abnormalities for up to 3 years before death, whereas wall thickness identified a population at risk only 18 to 24 months before death. CONCLUSIONS: Depressed LV FS and increased wall thickness are risk factors for mortality in HIV-infected children independent of depressed CD4 cell count and neurological disease. FS may be useful as a long-term predictor and wall thickness as a short-term predictor of mortality.

Assessing cardiac anatomy with digital subtraction angiography.

The use of intravenous digital subtraction angiography in the assessment of patients with congenital heart disease is described. Intravenous digital subtraction angiographic techniques are particularly helpful in visually assessing left to right shunts in patients with atrial and ventricular septal defects and anomalies of pulmonary venous return. Digital subtraction angiographic techniques have also been used to quantitate the magnitude of left to right shunts and have been compared with radionuclide-determined pulmonary flow/systemic flow ratios (r = 0.89, p less than 0.0001). Digital subtraction angiographic techniques are particularly helpful in evaluating the right atrium, right ventricle and pulmonary arteries as well as the aorta and aortic arch. We have used digital subtraction angiographic techniques in the immediate and long-term postoperative evaluations of patients after congenital heart surgery. The safety of digital subtraction angiography lends itself to pediatric patients and can be performed on an out patient basis. Digital subtraction angiography provides significant anatomic information that has modified our traditional approach to patients with congenital heart disease.

Association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations.

To determine the clinical, laboratory and hemodynamic profile in patients with primary pulmonary hypertension and associated portal hypertension, 7 new cases and 71 previously reported cases were analyzed. There was no gender predilection and the average age at diagnosis was 41 years. Liver cirrhosis was the most frequent cause of hypertension (82%) and a surgical portosystemic shunt was present in 29%. Almost invariably, portal hypertension either preceded or was diagnosed concurrently with pulmonary hypertension, favoring the hypothesis that in portal hypertension, the pulmonary vasculature may be exposed to vasoactive substances normally metabolized or produced by the diseased liver, possibly inducing vasoconstriction or direct toxic damage to the pulmonary arteries. Clinically, exertional dyspnea was the most frequent presenting symptom (81%); other symptoms, such as syncope, chest pain and fatigue, were present in less than 33%. An accentuated pulmonary component of the second heart sound (82%) and a systolic murmur (69%) were the most common physical findings. At least 75% of these patients had evidence of pulmonary hypertension on electrocardiography (right ventricular hypertrophy) or roentgenography (cardiomegaly or dilated main pulmonary arteries, or both). Hemodynamic findings included severe pulmonary hypertension (mean pulmonary artery pressure 59 +/- 19 mm Hg) with normal pulmonary capillary wedge pressure and cardiac output. Treatment was basically palliative and the mean and median survival times were 15 and 6 months, respectively. In brief, on the basis of clinical presentation and laboratory features, patients with combined primary pulmonary hypertension and portal hypertension seldom represent a diagnostic challenge. Further research is needed on treatment, which remains palliative. The survival rate is poor and worse than that seen in isolated primary pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

Patent ductus arteriosus in adults--long-term follow-up: nonsurgical versus surgical treatment.

The long-term prognosis of adult patients with patent ductus arteriosus treated medically or surgically has not been demonstrated. One hundred seventeen adult patients ranging in age from 18 to 81 years (mean 36) were followed up for 1 to 37 years (mean 18). Forty-five patients were treated nonsurgically and 72 had surgical closure. The nonsurgical group experienced significantly more cyanosis (p = 0.002) and had fewer diastolic murmurs (p greater than 0.001) than did the surgical group. In the nonsurgical group, patients with cardiomegaly before treatment were more likely to die (p greater than 0.001) than were patients who did not have cardiomegaly. In the surgical group, patients with cardiomegaly before treatment had a worse prognosis (p = 0.09) than that of patients with normal heart size. Seven patients, five in the nonsurgical and two in the surgical group, presented with a pulmonary artery systolic pressure greater than 100 mm Hg. Five of these patients are alive and well at a mean follow-up time of 18 years. The two nonsurvivors were in the nonsurgical group and died at 4 and 19 years of follow-up, respectively. Adult patients with patent ductus arteriosus should be treated surgically, especially if cardiomegaly is seen at initial presentation.

Task Force 4: The relationship between cardiovascular specialists and generalists.

1. An imbalance between generalists and cardiovascular subspecialists exists that will require decades to correct. We question the validity of the 50:50 generalist/specialist ratio in view of current practice patterns for cardiology. 2. There has been a large increase in the number of cardiovascular specialists in the past 30 years that will continue if training programs remain at their current size. 3. Cardiovascular specialists provide a substantial amount of inpatient care, care to older patients and care to those with cardiovascular symptoms, although generalists actually provide the majority of office-based cardiovascular care. 4. A significant portion of cardiovascular specialist care can be classified as comprehensive care to patients with and without cardiovascular disease. 5. Most generalists and cardiovascular specialists do not perceive a need for additional cardiovascular specialists. 6. Many providers perform cardiovascular procedures at levels below the recommended threshold for maintenance of clinical competence. 7. Managed care may result in a reduced demand for cardiovascular specialists. 8. If cardiovascular specialists provide general care, it may not be assumed that previous training prepares them for generalist practice. 9. The appropriate boundaries of cardiovascular care between generalists and cardiovascular specialists are indistinct. They are defined somewhat by the training programs from which the generalists graduate. 10. Many generalists have deficiencies in basic skills in clinical cardiology. 11. Desirable interactions between generalists and cardiovascular specialists involve referral of patients in both directions to the provider who can give care to a given patient, at a given time, with the best outcome and lowest cost.(ABSTRACT TRUNCATED AT 250 WORDS)

Intravenous digital subtraction angiography in the assessment of patients with left to right shunts before and after surgical correction.

Pre- and postoperative structural changes and pulmonary to systemic flow (QP/QS) ratios were assessed using digital angiography in 34 patients documented to have a left to right shunt at cardiac catheterization. There were 16 men and 18 women whose ages ranged from 4 months to 60 years. The radiographic single mask mode was used for all digital subtraction angiographic studies with a typical radiographic sequence being 80 to 100 kV, 5 to 10 mA/frame at six frames/s for 15 seconds. Renografin-76 was used as a bolus injection at 0.5 to 1.0 ml/kg via an arm vein in most patients. The level of the left to right shunt and any associated anomalies were noted and compared with results from cardiac catheterization. Digital subtraction angiographic flow curves were generated from the pulmonary arteries, and QP/QS ratios were calculated pre- and postoperatively using the gamma variate fit method and compared with the QP/QS ratio from first pass radionuclide studies. A strong correlation between preoperative digital subtraction angiographically derived QP/QS ratio and radionuclide-derived QP/QS ratio was found, with an r value equal to 0.89, p less than 0.0001. Postoperatively, all patients had a QP/QS ratio less than 1.2:1.0 for both digital subtraction angiography and radionuclide studies. The level of left to right shunt was accurately assessed in all patients, and its absence observed postoperatively. Associated anomalies, such as a persistent left superior vena cava, coarctation of the aorta and partial anomalous venous return, were identified in all cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Cystic medial necrosis of the aorta in patients without Marfan's syndrome: surgical outcome and long-term follow-up.

A retrospective analysis was performed to determine the surgical outcome and long-term follow-up of patients with documented cystic medial necrosis of the aorta. Ninety-three patients were diagnosed as having cystic medial necrosis at the Cleveland Clinic between July 1963 and December 1987 (72% men aged 26 to 77 years, mean 55). Patients who met the standard diagnostic criteria for Marfan's syndrome were deliberately excluded. Sixty-eight percent of the patients had a diastolic murmur and chest roentgenogram revealed a dilated aortic arch in 58% and cardiomegaly in 63%. Cardiac catheterization in 76 patients demonstrated aortic root dilation in 78%, aortic regurgitation in 72%, aortic dissection in 32% and coronary artery disease in 32%. Ninety patients underwent surgery including composite graft repair with reimplantation of the coronary arteries in 34%. Follow-up, obtained on 90 (97%) of the 93 patients, ranged in duration from 0 to 137 months (mean 29). Thirty-four of the 90 patients died (age range 30 to 75 years, mean 60). Ninety-four percent of the known causes of death were related to the cardiovascular system; 65% were the result of aortic dissection or rupture or sudden death. Ninety-six percent of survivors were in New York Heart Association functional class I or II. Overall estimated survival at 1, 3 and 5 years was 72.2%, 63.5% and 57.4%, respectively. Actuarial survival in patients who underwent composite graft reconstruction was 84% at 5 years. The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p = 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)

Marfan's syndrome: natural history and long-term follow-up of cardiovascular involvement.

A retrospective analysis was undertaken to define the natural history and long-term follow-up of a group of patients with Marfan's syndrome. Eighty-four patients were diagnosed between January 1959 and June 1987 as having Marfan's syndrome; 68% were male; their ages ranged from 2 to 67 years (mean 26.6). Sixteen patients constituted the early surgical group (those who underwent surgery before 1979; mean age 36.1 years). Nineteen patients constituted the late surgical group (surgery in 1979 or later; mean age 33.3 years). The nonsurgical group comprised 49 patients (mean age 19.3 years). Fifty-seven percent of the patients had a diastolic murmur and 38% had cardiomegaly at presentation. Fifty-seven percent underwent cardiac catheterization, which revealed aortic root dilation (85%), aortic regurgitation (73%), aortic dissection (33%) and mitral regurgitation (36%). Thirteen of the 19 patients in the late surgical group received a composite graft repair of the ascending aorta as compared with only 2 of the 16 in the early surgical group. Follow-up information was obtained on 81 (96%) of 84 patients; the follow-up time was 2 to 332 months (mean 99). Thirty-one of the 81 patients died at age 3 to 63 years (mean age 35 years); 87% of the known causes of death were related to the cardiovascular system. Sixty-one percent of deaths were the result of aortic dissection or rupture or sudden cardiac death. Of the 50 survivors, 98%, including all patients in the late surgical group, were in functional class I or II. Overall survival at 5, 10 and 15 years after operation was 78.4%, 57.1% and 49.5%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Prevalence of congenital cardiovascular malformations in children of human immunodeficiency virus-infected women: the prospective P2C2 HIV Multicenter Study. P2C2 HIV Study Group, National Heart, Lung, and Blood Institute, Bethesda, Maryland.

OBJECTIVES: The purpose of the study was to assess the effects of maternal HIV-1 (human immunodeficiency virus) infection and vertically transmitted HIV-1 infection on the prevalence of congenital cardiovascular malformations in children. BACKGROUND: In the United States, an estimated 7000 children are born to HIV-infected women annually. Previous limited reports have suggested an increase in the prevalence of congenital cardiovascular malformations in vertically transmitted HIV-infected children. METHODS: In a prospective longitudinal multicenter study, diagnostic echocardiograms were performed at 4-6-month intervals on two cohorts of children exposed to maternal HIV-1 infection: 1) a Neonatal Cohort of 90 HIV-infected, 449 HIV-uninfected and 19 HIV-indeterminate children; and 2) an Older HIV-Infected Cohort of 201 children with vertically transmitted HIV-1 infection recruited after 28 days of age. RESULTS: In the Neonatal Cohort, 36 lesions were seen in 36 patients, yielding an overall congenital cardiovascular malformation prevalence of 6.5% (36/558), with a 8.9% (8/90) prevalence in HIV-infected children and a 5.6% (25/449) prevalence in HIV-uninfected children. Two children (2/558, 0.4%) had cyanotic lesions. In the Older HIV-Infected Cohort, there was a congenital cardiovascular malformation prevalence of 7.5% (15/201). The distribution of lesions did not differ significantly between the groups. CONCLUSIONS: There was no statistically significant difference in congenital cardiovascular malformation prevalence in HIV-infected versus HIV-uninfected children born to HIV-infected women. With the use of early screening echocardiography, rates of congenital cardiovascular malformations in both the HIV-infected and HIV-uninfected children were five- to ten-fold higher than rates reported in population-based epidemiologic studies but not higher than in normal populations similarly screened. Potentially important subclinical congenital cardiovascular malformations were detected.

Cardiac structure and function in fetuses of mothers infected with HIV: the prospective PCHIV multicenter study.

BACKGROUND: This study was designed to determine if vertically transmitted HIV infection and maternal infection with HIV are associated with altered cardiovascular structure and function in utero. METHODS: Fetal echocardiography was performed in 173 fetuses of 169 HIV-infected mothers (mean gestational age, 33.0 weeks; SD = 3.7 weeks) at 5 centers. Biparietal diameter, femur length, cardiovascular dimensions, and Doppler velocities through atrioventricular and semilunar valves and the umbilical artery were measured. Measurements were converted to z scores based on published normal data. RESULTS: Fetuses determined after birth to be HIV-infected had similar echocardiographic findings as fetuses later determined to be HIV-uninfected except for slightly smaller left ventricular diastolic dimensions (P =.01). The femur length (P =.03) was also smaller in the fetuses postnatally identified as HIV-infected. Differences in cardiovascular dimensions and Doppler velocities were identified between fetuses of HIV-infected women and previously published normal fetal data. The reason for the differences may be a result of maternal HIV infection, maternal risk factors, or selection bias in the external control data. CONCLUSIONS: Vertically transmitted HIV infection may be associated with reduced left ventricular size but not with altered cardiac function in utero. Fetuses of HIV-infected mothers may have abnormal cardiovascular structure and function and increased placental vascular resistance, regardless of whether the fetuses are subsequently found to be infected with HIV.

Routine cholesterol surveillance in childhood.

Coronary heart disease is the leading cause of death in the United States, and there is reason to believe that it begins in childhood. Evidence is accumulating that early diagnosis and treatment of hypercholesterolemia, a major coronary risk factor, can markedly reduce the incidence of atherosclerotic heart disease in later life. A pediatric group practice consisting of six pediatricians and a pediatric nurse practitioner performed a cholesterol surveillance study of 6500 children between 3 and 18 years of age. Parents and patients were counseled regarding other coronary risk factors, and the American Heart Association diet was recommended. According to the results of the study, 1251 children (19%) exceeded the acceptable 90th percentile for cholesterol and that 143 of 299 significantly hypercholesterolemic children (48%) had no family history of premature myocardial infarction or known hypercholesterolemia. The current recommendation is that only those children from high-risk families should be screened for an elevated cholesterol level. The authors conclude, as a result of this study, that all children older than 3 years of age should have a cholesterol test and that advice regarding avoidance of high-risk coronary life-style behaviors should be a routine part of pediatric anticipatory guidance.

Thallium-20 1 myocardial imaging in young adults with anomalous left coronary artery arising from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery (Bland-Garland-White syndrome) may produce myocardial ischemia, infarction, and frequently death in infancy. Some patients, however, develop satisfactory coronary artery collaterals and are relatively asymptomatic into adulthood. Very little is known about their myocardial perfusion patterns. We studied three young adults with this condition using stress thallium-20 1 myocardial imaging. Electrocardiograms in two patients demonstrated old anterolateral myocardial infarctions. Preoperative stress exercise tests were positive in all three patients. Marked perfusion abnormalities were found in the proximal anterolateral wall in all patients, and one patient also had a posterolateral defect. Postoperatively, all stress tests returned to normal. Thallium imaging demonstrated improvement in ischemic areas, but old scars persisted.

Recirculation subtraction for analysis of left-to-right-cardiac shunts: concise communication.

The object of this study is to improve the techniques for describing the lung dilution curve for shunt quantification by separating the effects of systemic recirculation on the curve form those of direct shunt return. The time of the systemic recirculation peak was estimated by determination of transit times from the right and left ventricles and lung. A gamma variate fit based on the distribution of points at that segment was applied to the recirculation curve and subtracted from the original lung dilution curve. Similar gamma variate fitting was performed for both primary and shunt curves. Rather than fitting the gamma variate of the shunt curve by the leading edge only, a larger portion could now be used since the trailing edge of the curve is clearer following recirculation subtraction. The algorithm is completely automatic, requiring no operator intervention or selection of curve-fitting regions. The correlation coefficient for comparison of the dilution-curve analysis with oximetry determinations was 0.92 in a series of 29 patients.

Long-term follow-up in the unoperated univentricular heart.

Type A univentricular heart is a severe congenital cardiac defect; 50% of patients are dead 14 years after diagnosis, a death rate of 4.8% per year. Type C univentricular heart is even more lethal; 50% of patients are dead 4 years after diagnosis. Pulmonary stenosis did not protect either group of patients in terms of overall survival. Of the patients in class I or II at initial diagnosis, 34% of patients with type A and 12% of those with type C were in class III or IV or had died at follow-up. The most common causes of death were dysrhythmia, congestive heart failure and sudden and unexplained death.

Long-term follow-up after palliative operation for univentricular heart.

Data are presented from 84 patients (45 males, 39 females), aged 6 days to 25 years (mean 10 years), with univentricular heart who underwent a palliative surgical procedure. The mean follow-up period was 5 years (range 1 month to 25 years). Of the 84 patients, 33 died and 51 are alive. The survivors were 1.5 to 41 years (mean 15). Systemic pulmonary artery shunts alone were performed in 43 patients, pulmonary artery banding in 15, and a combination of procedures in 26. Five years after diagnosis, 70% of patients with type A and 54% with type C univentricular heart were alive, with no statistical difference in survival between the patient groups. Because of the high attrition rate in these patients despite palliation, further attempts to perfect corrective surgery appear justified.

Neonatal pneumopericardium.

Three cases of neonatal pneumopericardium are presented. Detailed ventilatory measurements and chest roentgenographic changes are described for each of the three patients. The literature of neonatal pneumopericardium is reviewed, and based on that review and our own cases, we offer guidelines for managing these patients.

Cor triatriatum: echocardiograhic findings.

The echocardiographic findings in a patient with cor triatriatum are presented. The preoperative echocardiogram demonstrated an unusual structure behind the mitral valve that moved anteriorly during atrial systole. There was also echocardiographic evidence of abnormalities of mitral valve motion, right ventricular and left atrial enlargement, and pulmonary hypertension. Postoperatively, the unusual echo behind the mitral valve had disappeared and the mitral valve appeared more normal. Left atrial and right ventricular dimension had decreased and the pulmonary valve appeared more normal. Fine diastolic mitral valve fluttering, an abnormal echo behind the anterior mitral valve leaflet moving anteriorly with atrial systole, an abnormal pulmonary valve echogram suggesting pulmonary hypertension, and marked right ventricular and left atrial enlargement were the main echocardiographic findings in our patient.

Malignant primary cardiac tumors. The Cleveland Clinic experience, 1956 to 1986.

Malignant primary cardiac tumors have uniformly been associated with poor long-term survival. We reviewed data on 11 patients with such tumors seen between 1956 and 1986. There were five men and six women, with a mean age at presentation of 44 years. Nine (82 percent) of 11 patients presented initially with respiratory symptoms, and 45 percent (five) presented with chest pain. No patient was asymptomatic on initial presentation. Forty-five percent (five) of the patients had elevated jugular venous pressure or a systolic murmur. Cardiomegaly was present in eight (73 percent) of 11 patients. Nine of ten patients had a hemoglobin level less than 12 g/100 ml, and 30 percent (3/10) presented with thrombocytosis. Seventy percent (eight) of the patients underwent surgical biopsy, and in 30 percent (three), surgical excision of the tumor was attempted. Angiosarcoma was the most frequent type of tumor (four patients), followed by malignant fibrous histiocytoma (three patients), mesothelioma (two patients), rhabdomyosarcoma (one patient), and primary lymphoma (one patient). Long-term follow-up data reveals that ten (91 percent) of 11 patients had died, with a mean duration from diagnosis until death of 9.7 months. Newer pathologic techniques for identification of sarcomas, aggressive surgical resection, and advanced chemotherapy for certain subgroups may contribute to better survival for patients with these tumors in the future. In addition, cardiac transplantation may play an important role in the future for those patients with localized disease.

Staged surgical management of pulmonary atresia with diminutive pulmonary arteries.

A particular problem in treatment is presented by patients who have atresia of the pulmonary valve and ventricular septal defect but whose pulmonary arterial tree is extremely diminutive. The pulmonary blood flow results from naturally occurring large bronchial collateral arteries. In the experience reported, satisfactory palliation was provided for all 3 patients in whom right ventricle--to--pulmonary artery continuity was constructed by placing a pericardial patch graft during open-heart operation. The ventricular septal defect was left unrepaired. Early evidence supports the hypothesis that, besides providing palliation of cyanosiis, the procedure may allow progressive enlargment of the hypoplastic pulmonary arteries. This offers hope that later second-stage completion of total repair, involving closure of the ventricular septal defect and ligation of any remaining large bronchial collateral arteries, may become feasible.