Douglas Moodie

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)

Anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome) in adult patients: Long-term follow-up after surgery

Congenital anomalous origin of the left coronary artery arising from the pulmonary artery, or the Bland-White-Garland syndrome (BWGS), is an uncommon but frequently lethal lesion of both children and adults.’ Although approximately 80 % to 90% of patients develop congestive heart failure and die in infancy, some patients may present as adolescents or adults.*z3 Although often asymptomatic, many adults with BWGS demonstrate evidence of previous myocardial infarction and frequently exhibit ischemic patterns when evaluated with stress electrocardiography or stress thallium-201 myocardial imaging.4-6 Several reports of recurrent arrhythmias7 or sudden death2s8 in such patients have been published. Most clinicians recognize the need for surgical intervention. Surgical approaches have included reimplantation of the anomalous left coronary artery from the pulmonary artery to the aorta,g ligation of the anomalous left coronary artery from inside the pulmonary artery,‘O or ligation of the left coronary artery combined with saphenous vein grafting to the anterior descending coronary artery.” No data are currently available which demonstrate the superiority of one surgical approach over another in adult patients. This report describes the presentation, evaluation, and long-term follow-up in 10 adult patients with BWGS following various methods of surgical repair.

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.

Adult congenital heart disease

Much of the literature published in 1993 on the topic of adult congenital heart disease described long-term follow-up of patients following repair in childhood. This report reviews the most important papers published during the past year related to long-term follow-up, and also describes new information on the noninvasive evaluation of congenital heart disease in adults as well as the interventional cardiologic techniques that are currently being applied to treat some defects in the catheterization laboratory.

Outcomes of Heart Failure–Related Hospitalization in Adults with Congenital Heart Disease in the United States

BACKGROUND Heart failure (HF) accounts for >3 million hospital admissions annually in adults with acquired cardiovascular disease, but there are limited data on HF admissions in adults with congenital heart disease (ACHD). The purpose of this study was to test the hypotheses that HF admissions are common in ACHD and associated with significant morbidity and mortality. METHODS The 2007 Nationwide Inpatient Sample was used to assess national prevalence, morbidities, and risk factors for mortality during hospitalizations among ACHD with HF. RESULTS Of the 84,308 (95% CI 71,345-97,272) ACHD admissions in the United States in 2007, 17,193 (95% CI 14,157-20,229) had a diagnosis of HF (20%). ACHD with HF was associated with an increased risk of death compared to ACHD without HF (OR 3.3, 95% CI 2.6-4.1). On multivariable analysis independent risk factors for mortality included nonoperative intubation (OR 6.1, 95% CI 3.3-11.4), sepsis (OR 4.3, 95% CI 2.4-7.4), and acute myocardial infarction (OR 3.2, 95% CI 1.8-5.7). Cardiac defects associated with an increased risk of mortality included ventricular septal defects (VSDs) (OR 1.8, 95% CI 1.0-3.4). CONCLUSIONS In this large population-based study, HF-related hospitalizations were common in ACHD and associated with an increased risk of death compared to non-HF admissions. The risk of mortality is increased with the diagnoses of VSDs and the presence of specific comorbidities such as respiratory failure and sepsis.

Cardiac Dysfunction and Mortality in HIV-Infected Children

BackgroundLeft 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 ResultsBaseline 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 (P ≤0.02 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. ConclusionsDepressed 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.

Left Ventricular Structure and Function in Children Infected With Human Immunodeficiency Virus

BACKGROUND The frequency of, course of, and factors associated with cardiovascular abnormalities in pediatric HIV are incompletely understood. METHODS AND RESULTS A baseline echocardiogram (median age, 2.1 years) and 2 years of follow-up every 4 months were obtained as part of a prospective study on 196 vertically HIV-infected children. Age- or body surface area-adjusted z scores were calculated by use of data from normal control subjects. Although 88% had symptomatic HIV infection, only 2 had CHF at enrollment, with a 2-year cumulative incidence of 4.7% (95% CI, 1.5% to 7.9%). All mean cardiac measurements were abnormal at baseline (decreased left ventricular fractional shortening [LV FS] and contractility and increased heart rate and LV dimension, mass, and wall stresses). Most of the abnormal baseline cardiac measurements correlated with depressed CD4 cell count z scores and the presence of HIV encephalopathy. Heart rate and LV mass showed significantly progressive abnormalities, whereas FS and contractility tended to decline. No association was seen between longitudinal changes in FS and CD4 cell count z score. Children who developed encephalopathy during follow-up had depressed initial FS, and FS continued to decline during follow-up. CONCLUSIONS Subclinical cardiac abnormalities in HIV-infected children are common, persistent, and often progressive. Dilated cardiomyopathy (depressed contractility and dilatation) and inappropriate LV hypertrophy (elevated LV mass in the setting of decreased height and weight) were noted. Depressed LV function correlated with immune dysfunction at baseline but not longitudinally, suggesting that the CD4 cell count may not be a useful surrogate marker of HIV-associated LV dysfunction. However, the development of encephalopathy may signal a decline in FS.

Outcomes of Hospitalization in Adults in the United States With Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect

Atrial septal defect, ventricular septal defect (VSD), and atrioventricular septal defect (AVSD) are among the most common congenital heart lesions, with most children surviving to adulthood. However, the clinical course of these patients is largely unknown, particularly pertaining to inpatient care. The purpose of this study was to assess hospitalizations for septal defects in adults with congenital heart disease (CHD) and risk factors associated with significant morbidity and mortality. The 2007 Nationwide Inpatient Sample was used to assess national prevalence of hospitalizations in adults with CHD with septal defects. Co-morbidities and risk factors for mortality were also determined. There were 84,308 adult CHD admissions in the United States in 2007. Fifty-four percent of adult CHD admissions had diagnoses of septal defects, with 48% having atrial septal defect, 7% having VSD, and 0.4% having AVSD. Overall in-hospital mortality was 2.1%. Common co-morbidities included arrhythmias (31%), heart failure (20%), and diabetes mellitus (18%). On multivariable analysis, independent risk factors for mortality included presence of VSD (odds ratio 3.1, 95% confidence interval [CI] 1.5 to 6.5), trisomy 21 (odds ratio 2.9, 95% CI 1.1 to 7.5), and pulmonary hypertension (odds ratio 1.5, 95% CI 1.0 to 2.4). In conclusion, this study of hospitalizations in adults with septal defects found that admissions are common and associated with significant co-morbidities. Overall mortality is low but is increased in patients with VSD. Cardiac and noncardiac co-morbidities are commonly encountered. Many noncardiac conditions, including trisomy 21 and the youngest and oldest groups, are associated with an increased risk of death.

Narcotic antagonist therapy of the obesity hypoventilation syndrome.

A child with respiratory failure and cor pulmonale secondary to the obesity hypoventilation syndrome (OHS) was found to have abnormal β-endorphin levels in cerebrospinal fluid (CSF) and serum. A single iv dose of 10 μg/kg of naloxone early in the course of respiratory failure resulted in dramatic improvement which lasted approximately 3 to 4 h. The patient failed to respond to progesterone, and because of deteriorating respiratory status a low-dose continuous infusion of naloxone, 2μg/kg h, was begun and gradually increased to 10 μg/kg h, during which time there was a dramatic improvement in respiratory status and clinical condition. After 5 days, naloxone infusion was discontinued and progressive respiratory deterioration recurred. The child died of overwhelming sepsis and disseminated intravascular coagulation.

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)