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A Guide for Pediatric Pulmonary Hypertension

A Guide for Pediatric Pulmonary Hypertension

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and children. The disease affects nearly two of every 1,000 babies born each year in the United States, and children can suffer with health issues throughout their lives or die prematurely. However, with a proper diagnosis, rigorous evaluation, and comprehensive treatment, the prognosis for many of these children can be excellent, depending on the underlying cause of the pulmonary hypertension. The American Heart Association and American Thoracic Society convened a panel of experts to review the current literature and make recommendations on diagnosing, evaluating, and treating pediatric pulmonary hypertension. Published in Circulation and available for free online at http://circ.ahajournals.org, the guidelines are the result of extensive literature reviews of over 600 studies, extensive discussions, and formal scoring of recommendations. “These first-ever guidelines were rigorously developed in collaboration with a group of diverse experts that cross the traditional silos of several disciplines,” says Steven H. Abman, MD, the guidelines committee co-chair. “To optimize the care of pediatric patients with pulmonary hypertension, increased awareness of at-risk patients and the use of multidisciplinary care teams are critical.”   Key Highlights According to Dr. Abman, the guidelines provide clinicians with practical advice on classifying the many types of pulmonary hypertension, a key step in determining treatment. The document also discusses proven and emerging medical and surgical therapies as well as treatments that are approved for children with pulmonary hypertension and which drugs and dosages should be used. In addition, guidance is provided for long-term monitoring.   Specialized Care “It’s important to note that the causes, clinical presentations, and treatment...
Improving Pulmonary Hypertension Care

Improving Pulmonary Hypertension Care

The success of treatment for pulmonary hypertension (PH), including pulmonary arterial hypertension (PAH), is dependent on early detection and an accurate diagnosis. “PH treatments are typically directed toward the underlying cause, but there can be overlap between various patient groups,” says Mardi Gomberg-Maitland, MD, MSc. “This overlap can complicate diagnostic approaches, therapeutic choices, and anticipated outcomes.” Patients are often referred to PH centers with incomplete evaluations. “PH centers offer important benefits to patients because they have clinicians and nurse teams who are dedicated and trained in caring for these patients,” says Dr. Gomberg-Maitland. “Unfortunately, patients are often referred late in the course of illness. Some are given PAH-specific medications without appropriate testing or indications.” Furthermore, PAH medications are expensive and difficult to manage. Data on Pulmonary Hypertension Diagnosis & Referral In JAMA Internal Medicine, Dr. Gomberg-Maitland, Cherylanne Glassner-Kolmin, BS, and colleagues had a study published that assessed PH diagnosis accuracy in patients referred to PH centers. “We also wanted to look at the appropriateness of use of PAH-specific medications based on current guidelines,” says Glassner-Kolmin. According to findings, 68% of patients were referred by cardiologists or pulmonologists to PH centers and 61% had late-stage disease. One-third of patients who received a definitive PAH diagnosis before their referral received a misdiagnosis. “We also found that many patients were treated contradictorily to published recommendations, either having not undergone a heart catheterization, having been inappropriately prescribed medications, or both,” says Glassner-Kolmin. Dr. Gomberg-Maitland says major efforts have been made to educate medical professionals about PH, but misdiagnoses and inappropriate treatment are still occurring. “Inexperienced clinicians should be cautious of giving patients a...

Pulmonary Hypertension Remains After Aortic Valve Replacement

Most patients who undergo aortic valve replacement (AVR) appear to have pulmonary hypertension that remains following AVR, according to a Cleveland Clinic investigation. Any improvement in initial right ventricular systolic pressure after AVR returned to preoperative levels when reassessed at 3 to 4 years. Abstract: Journal of Thoracic and Cardiovascular Surgery, November...

A Look at Near-Miss Mortality and Morbidity in Pregnancy

Previous research suggests that timely delivery of appropriate healthcare services may prevent almost half of all pregnancy-related deaths in the United States. Although maternal deaths are increasingly attributed to preexisting condi­tions, the relative effect of these conditions and the extent to which maternal morbidity and mortality are concentrated in high-risk patients have not been well defined. Focusing on Near-Miss Pregnancy Events Jill M. Mhyre, MD, and colleagues had a study published in the November 2011 issue of Anesthesiology that sought to iden­tify which preexisting conditions predicted maternal morbidity, mortality, and “near- miss” events. A near miss occurs when preg­nant women or recently postpartum women survive a life-threatening event, either because of high-quality medical care or by chance alone. “In our study, we investigated women who died as well as those who sur­vived an event in the hopes of uncovering important lessons with regard to predicting outcomes and preventing progression from near misses to death,” says Dr. Mhyre. A near miss was defined as end-organ injury that impacted hospital discharge. The highest rates of near-miss morbidity or mortality events were found among women with pulmonary hypertension (PH), malig­nancy, and systemic lupus erythematosus (Table). Advancing maternal age and non-white race also increased risk for near-miss morbidity or mortality. “While the effect sizes we observed for age and race were relatively modest when compared with certain medical conditions, these effects remained statistically significant even after controlling for all other medical and obstet­ric conditions,” Dr. Mhyre says. “The abso­lute rate per thousand deliveries increased three-fold when age older than 40 was com­pared with ages 20 to 34, but the adjusted odds ratio was...

Conference Highlights: ASE 2012

New research was presented at ASE 2012, the American Society of Echocardiography’s 23rd Annual Scientific Sessions, from June 30 to July 3 in Maryland. The features below highlight just some of the studies that emerged from the conference. Echocardiograms for Diagnosing Pulmonary Hypertension The Particulars: Patients with stable heart failure who have high pulmonary artery systolic pressure are at increased risk for adverse outcomes. A reliable method is needed for measuring pulmonary artery systolic pressure in this patient population. Data Breakdown: Emory University researchers used echocardiography to diagnose pulmonary hypertension— defined as pulmonary artery systolic pressure higher than 45 mm Hg—in stable outpatients with heart failure. Echocardiography was found to strongly predict higher risk of clinical events. The testing also helped determine which patients would have higher hospitalization rates. Take Home Pearl: Pulmonary artery systolic pressure as measured by echocardiography appears to provide important prognostic information for patients with stable heart failure. Ultrasonography Helps Predict Atherosclerosis The Particulars: The incidence of peripheral arterial disease is rising throughout the United States. However, data from large population-based samples on the prevalence of subclinical atherosclerosis in the peripheral arteries are lacking. Data Breakdown: Investigators in a study used ultrasound exams to look for the presence and degree of popliteal arterial plaque as a predictor of atherosclerosis. Popliteal artery atherosclerosis was prevalent among patients aged 40 or younger who were at risk for becoming obese and/ or having diabetes. Popliteal artery atherosclerosis was independently associated with older age and albuminuria. Urinary albuminuria was also linked with the severity of atherosclerotic plaque burden in the popliteal arteries. Take Home Pearl: Among younger patients with...
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