Management of Pulmonary Hypertension From Left Heart Disease in Candidates for Orthotopic Heart Transplantation

Pulmonary hypertension in left heart disease (PH-LHD) commonly complicates prolonged heart failure (HF). When advanced, the PH becomes fixed or out of proportion and is associated with increased morbidity and mortality in patients undergoing orthotopic heart transplant (OHT). To date, the only recommended treatment of out of proportion PH is the treatment of the underlying HF by reducing the pulmonary capillary wedge pressure (PCWP) with medications and often along with use of mechanical circulatory support. Medical therapies typically used in the treatment of World Health Organization (WHO) group 1 pulmonary arterial hypertension (PAH) have been employed off-label in the setting of PH-LHD with varying efficacy and often negative outcomes. We will discuss the current standard of care including treating HF and use of mechanical circulatory support. In addition, we will review the studies published to date assessing the efficacy and safety of PAH medications in patients with PH-LHD being considered for OHT

Active Implantable cardioverter-defibrillators in Continuous-flow Left Ventricular Assist Device Recipients

Introduction Implantable cardioverter-defibrillator (ICD) in patients with heart failure with reduced ejection fraction reduces mortality secondary to malignant arrhythmias. Whether end-stage heart failure (HF) with continuous-flow left ventricular assist device (cf-LVAD) derive similar benefits remains controversial. Methods We performed a systematic literature review and meta-analysis of all published studies that examined the association between active ICDs and survival in advanced HF patients with cfLVAD. We searched PubMed, Medline, Embase, Ovid, and Cochrane for studies reporting the association between ICD and all-cause mortality in advanced HF patients with cfLVAD. Mantel-Haenszel risk ratio (RR) random-effects model was used to summarize data. Results Ten studies (9 retrospective and one prospective) with a total of 7,091 patients met inclusion criteria. There was no difference in all-cause mortality (RR 0.84, 95% CI 0.65–1.10, p=0.20, I2 =62.40%), likelihood of survival to transplant (RR 1.07, 95% CI 0.98–1.17, p= 0.13, I2 =0%), RV failure (RR 0.74, 95% CI 0.44–1.25, p = 0.26, I2 =34%) between Active ICD and inactive/no ICD groups, respectively. Additionally, 27.5% received appropriate ICD shocks, while 9.5% received inappropriate ICD shocks. No significant difference was observed in terms of any complications between the two groups. Conclusions All-cause mortality, the likelihood of survival to transplant, and worsening RV failure were not significantly different between active ICD and inactive/no ICD in cf-LVAD recipients. A substantial number of patients received appropriate ICD shocks suggesting a high-arrhythmia burden. The risks and benefits of ICDs must be carefully considered in patients with cf-LVAD

Hyperbaric treatment for children with autism: a multicenter, randomized, double-blind, controlled trial

<p>Abstract</p> <p>Background</p> <p>Several uncontrolled studies of hyperbaric treatment in children with autism have reported clinical improvements; however, this treatment has not been evaluated to date with a controlled study. We performed a multicenter, randomized, double-blind, controlled trial to assess the efficacy of hyperbaric treatment in children with autism.</p> <p>Methods</p> <p>62 children with autism recruited from 6 centers, ages 2–7 years (mean 4.92 ± 1.21), were randomly assigned to 40 hourly treatments of either hyperbaric treatment at 1.3 atmosphere (atm) and 24% oxygen ("treatment group", n = 33) or slightly pressurized room air at 1.03 atm and 21% oxygen ("control group", n = 29). Outcome measures included Clinical Global Impression (CGI) scale, Aberrant Behavior Checklist (ABC), and Autism Treatment Evaluation Checklist (ATEC).</p> <p>Results</p> <p>After 40 sessions, mean physician CGI scores significantly improved in the treatment group compared to controls in overall functioning (p = 0.0008), receptive language (p < 0.0001), social interaction (p = 0.0473), and eye contact (p = 0.0102); 9/30 children (30%) in the treatment group were rated as "very much improved" or "much improved" compared to 2/26 (8%) of controls (p = 0.0471); 24/30 (80%) in the treatment group improved compared to 10/26 (38%) of controls (p = 0.0024). Mean parental CGI scores significantly improved in the treatment group compared to controls in overall functioning (p = 0.0336), receptive language (p = 0.0168), and eye contact (p = 0.0322). On the ABC, significant improvements were observed in the treatment group in total score, irritability, stereotypy, hyperactivity, and speech (p < 0.03 for each), but not in the control group. In the treatment group compared to the control group, mean changes on the ABC total score and subscales were similar except a greater number of children improved in irritability (p = 0.0311). On the ATEC, sensory/cognitive awareness significantly improved (p = 0.0367) in the treatment group compared to the control group. Post-hoc analysis indicated that children over age 5 and children with lower initial autism severity had the most robust improvements. Hyperbaric treatment was safe and well-tolerated.</p> <p>Conclusion</p> <p>Children with autism who received hyperbaric treatment at 1.3 atm and 24% oxygen for 40 hourly sessions had significant improvements in overall functioning, receptive language, social interaction, eye contact, and sensory/cognitive awareness compared to children who received slightly pressurized room air.</p> <p>Trial Registration</p> <p>clinicaltrials.gov NCT00335790</p

Peripartum cardiomyopathy: definition, incidence, etiopathogenesis, diagnosis, and management.

Peripartum cardiomyopathy (PPCM) is a serious pregnancy-associated disorder of unknown etiology. The precise cellular and molecular mechanisms underlying PPCM are unclear. A heightened awareness among health care providers can result in early diagnosis of heart failure in late pregnancy and the early postpartum period. Though the symptoms of dyspnea and fatigue can result from normal physiologic changes during pregnancy, an electrocardiogram and brain natriuretic peptide level should be obtained in these patients, in addition to baseline laboratory tests such as a complete blood count, and basic metabolic and hepatic function panels. If the electrocardiogram and brain natriuretic peptide level are abnormal, an echocardiogram should be obtained. The role of endomyocardial biopsy for the diagnosis of PPCM is controversial. Patients should be started on diuretics if volume overloaded, and beta-blockers (preferably metoprolol) if no contraindications exist; angiotensin-converting enzyme inhibitors and angiotensin receptor blockers should be avoided during pregnancy or lactation. There are no standard, universally accepted guidelines for the management of PPCM. Although experimental therapies like bromocriptine, pentoxifylline and immunoglobulins have shown promising results, large double-blind randomized trials are essential to confirm the results of smaller studies. In patients with persistent severe left ventricular (LV) dysfunction, advanced therapies like mechanical circulatory support and heart transplantation should be considered. Owing to recent data demonstrating deterioration of LV systolic function after initial recovery, it is essential to maintain long-term follow up of these patients regardless of initial recovery of LV function. We present a comprehensive review of the literature etiopathogenesis, diagnosis, and management of PPCM