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The effect of exercise training in symptomatic patients with grown-up congenital heart disease: a review.

Expert Rev Cardiovasc Ther. 2018 Apr 30;:

Authors: Hooglugt JQ, van Dissel AC, Blok IM, de Haan FH, Jørstad HT, Bouma BJ, Mulder BJ, Winter MM

INTRODUCTION: The number of grown-up congenital heart disease (GUCH) patients is steadily increasing. Unfortunately, the majority of these patients suffer from late sequelae, with heart failure being the most common cause of death. Exercise training is beneficial and safe in patients with acquired heart failure, as well as in asymptomatic GUCH patients. However, its effect remains unknown in symptomatic GUCH patients. This could cause reticence on positive sports advice, with possible counterproductive effects. Areas covered: A review of current literature was performed to evaluate the effect of exercise training in symptomatic (NYHA≥2) GUCH patients. The search yielded a mere three studies including symptomatic patients, and another six studies including also patients in NYHA 1 without making clear distinction between the NYHA subgroups. Expert commentary: Suboptimal trial designs, low patient numbers, and homogeneity of investigated cardiac anomalies make this review insufficient to draw definite conclusions. However, all studies describe overall positive effects of exercise training in symptomatic GUCH patients in terms of exercise capacity and quality of life. There were no safety concerns. Larger-scaled, randomized controlled trials are needed to obtain certainty.

PMID: 29707966 [PubMed - as supplied by publisher]

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A case of cardiopulmonary arrest due to spontaneous coronary artery dissection in a pregnant woman.

BMJ Case Rep. 2017 Aug 16;2017:

Authors: Ejima E, Murasato Y

We present the case of a young pregnant woman with cardiopulmonary arrest due to acute coronary syndrome. Emergent coronary angiography (CAG) and intravascular ultrasound (IVUS) showed extensive coronary artery dissection in the left anterior descending artery, which was treated with primary percutaneous coronary intervention. After managing the heart failure and disseminated intravascular coagulation, a dead fetus was delivered via caesarean section 4 days after admission to the hospital. Follow-up CAG and IVUS at 18 months showed persistent dissection in the non-stented site; hence, another stent was implanted. Dual antiplatelet therapy was discontinued 6 months later; however, aspirin and beta-blockers were continued lifelong.

PMID: 28814587 [PubMed - indexed for MEDLINE]

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Alcohol Screening and Brief Intervention: A Potential Role in Cancer Prevention for Young Adults.

Am J Prev Med. 2017 Sep;53(3S1):S55-S62

Authors: McKnight-Eily LR, Henley SJ, Green PP, Odom EC, Hungerford DW

Excessive or risky alcohol use is a preventable cause of significant morbidity and mortality in the U.S. and worldwide. Alcohol use is a common preventable cancer risk factor among young adults; it is associated with increased risk of developing at least six types of cancer. Alcohol consumed during early adulthood may pose a higher risk of female breast cancer than alcohol consumed later in life. Reducing alcohol use may help prevent cancer. Alcohol misuse screening and brief counseling or intervention (also called alcohol screening and brief intervention among other designations) is known to reduce excessive alcohol use, and the U.S. Preventive Services Task Force recommends that it be implemented for all adults aged ≥18 years in primary healthcare settings. Because the prevalence of excessive alcohol use, particularly binge drinking, peaks among young adults, this time of life may present a unique window of opportunity to talk about the cancer risk associated with alcohol use and how to reduce that risk by reducing excessive drinking or misuse. This article briefly describes alcohol screening and brief intervention, including the Centers for Disease Control and Prevention's recommended approach, and suggests a role for it in the context of cancer prevention. The article also briefly discusses how the Centers for Disease Control and Prevention is working to make alcohol screening and brief intervention a routine element of health care in all primary care settings to identify and help young adults who drink too much.

PMID: 28818247 [PubMed - indexed for MEDLINE]

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An Observation from Liver Biopsies Two Decades Post-Fontan.

Pediatr Cardiol. 2016 Aug;37(6):1119-22

Authors: Evans WN, Acherman RJ, Reardon LC, Galindo A, Rothman A, Ciccolo ML, Carrillo SA, Winn BJ, Yumiaco NS, Restrepo H

This brief report describes an observation from liver biopsy results in nonfailing Fontan patients, currently in their second postoperative decade. In three patients, with either atriopulmonary or atrioventricular connections and functional left ventricles, we found no portal fibrosis. In contrast, we found portal fibrosis in three clinically similar, nonfailing Fontan patients with lateral tunnel connections and functional left ventricles. We recognize the results may be secondary to chance; nevertheless, we speculate about possible relevancy.

PMID: 27160101 [PubMed - indexed for MEDLINE]

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Dilatation and Dysfunction of the Neo-aortic Root and in 76 Patients After the Ross Procedure.

Pediatr Cardiol. 2016 Aug;37(6):1175-83

Authors: Zimmermann CA, Weber R, Greutmann M, Dave H, Müller C, Prêtre R, Seifert B, Buechel EV, Kretschmar O, Attenhofer Jost CH

Pulmonary autograft replacement (Ross procedure) is used as an alternative to prosthetic aortic valve replacement patients with aortic valve disease. There are limited data on incidence and risk factors for dilatation and dysfunction of the neo-aortic after the Ross procedure. Ross procedure was performed in 100 patients at our institution between 1993 and 2011. In 76 patients, complete follow-up data were available. Their median age at surgery was 16 (0.4-58) years (76 % males; 95 % with congenital aortic valve disease). Median follow-up duration was 5.2 years (0.3-16.0 years). We analyzed their clinical and echocardiographic follow-up to identify possible risk factors for neo-aortic root dilatation and dysfunction. Ross procedure included reduction plasty of the native ascending aorta in 25 % of patients. During follow-up, 21 patients (28 %) developed neo-aortic root dilatation, 38 patients (50 %) dilatation oft the native ascending aorta and 7 patients (9 %) at least moderate neo-aortic regurgitation. Univariate risk factors for neo-aortic root dilatation were preoperative aortic regurgitation (p = 0.04), concomitant reduction plasty of the ascending aorta (p = 0.009) and a longer duration of follow-up (p = 0.005). Younger age at surgery was associated with dilatation of the ascending aorta (p = 0.03). Reoperation on the neo-aortic root because of severe dilatation was necessary in 6 patients (8 %), where 2 patients had at least moderate neo-aortic root regurgitation. Neo-aortic root and aortic dilatation are common after the Ross procedure. This is often combined with neo-aortic valve dysfunction. Close follow-up of these patients is mandatory.

PMID: 27300557 [PubMed - indexed for MEDLINE]

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Outcomes Following Melody Transcatheter Pulmonary Valve Implantation for Right Ventricular Outflow Tract Dysfunction in Repaired Congenital Heart Disease: First Reported Australian Single Centre Experience.

Heart Lung Circ. 2017 Oct;26(10):1085-1093

Authors: Markham R, Challa A, Kyranis S, Nicolae M, Murdoch D, Savage M, Malpas T, Radford DJ, Hamilton-Craig C, Walters DL

BACKGROUND: Transcatheter pulmonary valve implantation (TPVI) with the Melody® transcatheter pulmonary valve (TPV) has demonstrated good haemodynamic and clinical outcomes in the treatment of right ventricular outflow tract (RVOT) conduit dysfunction in patients with repaired congenital heart disease CHD. We present the first Australian single centre experience of patients treated with Melody TPV.
METHOD: A prospective, observational registry was developed to monitor clinical and haemodynamic outcomes in patients with RVOT dysfunction treated with the Melody TPV (Medtronic Inc, Minneapolis, United States).
RESULTS: Seventeen patients underwent TPVI with Melody TPV at The Prince Charles Hospital between January 2009 and February 2016 with a median (range) age of 34 (R: 15-60). Fifteen (88%) were NYHA Class 2 dyspnoea and 11 (59%) had corrected Tetralogy of Fallot. Indication for TPVI was stenosis in eight (47%), regurgitation in two (12%) and mixed dysfunction in seven (41%). Device implantation was successful in all patients. Peak RVOT gradient was significantly reduced and there was no significant regurgitation post procedure. There was one (6%) major procedural adverse event and two (12%) major adverse events at last recorded follow-up. There were no patient deaths. Follow-up cardiac magnetic resonance imaging revealed a significant reduction in indexed right ventricular end diastolic volume.
CONCLUSION: This study confirms the safety and effectiveness of TPVI with Melody TPV for RVOT dysfunction in repaired CHD.

PMID: 28185817 [PubMed - indexed for MEDLINE]

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Treatment of Myocardial Infarction and Mitral Regurgitation in a Patient with Congenitally Corrected Transposition of the Great Arteries.

J Heart Valve Dis. 2016 11;25(6):742-744

Authors: Mu JS, Cheng Jianqun Zhang B, Bo P

Congenitally corrected transposition of the great arteries (CTGA) is a rare congenital heart disease. In patients with functional CTGA with circumflex artery occlusion and mitral regurgitation (MR), the right ventricle functions as the left ventricle. Coronary artery bypass grafting with mitral valve replacement is an effective treatment for CTGA with concomitant myocardial infarction (MI) and MR.

PMID: 28290175 [PubMed - indexed for MEDLINE]

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Effect of maternal age and cardiac disease severity on outcome of pregnancy in women with congenital heart disease.

Int J Cardiol. 2017 Sep 15;243:197-203

Authors: Furenäs E, Eriksson P, Wennerholm UB, Dellborg M

BACKGROUND: There is an increasing prevalence of women with congenital heart defects reaching childbearing age. In western countries women tend to give birth at a higher age compared to some decades ago. We evaluated the CARdiac disease in PREGnancy (CARPREG) and modified World Health Organization (mWHO) risk classifications for cardiac complications during pregnancies in women with congenital heart defects and analyzed the impact of age on risk of obstetric and fetal outcome.
METHODS: A single-center observational study of cardiac, obstetric, and neonatal complications with data from cardiac and obstetric records of pregnancies in women with congenital heart disease. Outcomes of 496 pregnancies in 232 women, including induced abortion, miscarriage, stillbirth, and live birth were analyzed regarding complications, maternal age, mode of delivery, and two risk classifications: CARPREG and mWHO.
RESULTS: There were 28 induced abortions, 59 fetal loss, 409 deliveries with 412 neonates. Cardiac (14%), obstetric (14%), and neonatal (15%) complications were noted, including one maternal death and five stillbirths. The rate of cesarean section was 19%. Age above 35years was of borderline importance for cardiac complications (p=0.054) and was not a significant additional risk factor for obstetric or neonatal complications. Both risk classifications had moderate clinical utility, with area under the curve (AUC) 0.71 for CARPREG and 0.65 for mWHO on cardiac complications.
CONCLUSIONS: Pregnancy complications in women with congenital heart disease are common but severe complications are rare. Advanced maternal age does not seem to affect complication rate. Existing risk classification systems are insufficient in predicting complications.

PMID: 28539207 [PubMed - indexed for MEDLINE]

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Elevated sympathetic activity, endothelial dysfunction, and late hypertension after repair of coarctation of the aorta.

Int J Cardiol. 2017 Sep 15;243:185-190

Authors: Lee MGY, Hemmes RA, Mynard J, Lambert E, Head GA, Cheung MMH, Konstantinov IE, Brizard CP, Lambert G, d'Udekem Y

BACKGROUND: There is a high prevalence of late hypertension after coarctation repair. The relative contribution of elevated sympathetic tone and endothelial dysfunction to its development is unknown. This study aims to investigate the neural profile of coarctation patients including muscle sympathetic nerve activity testing to directly measure sympathetic nervous activity.
METHODS: Twenty-three patients aged ≥18years with a coarctation repair underwent measurements of clinic and 24-h blood pressures, muscle sympathetic nerve activity, sympathetic and cardiac baroreflex functions, digital endothelial function, and ambulatory arterial stiffness index. Median age at repair was 1.2months (interquartile range: 0-9months). Patients were compared to 17 healthy matched controls.
RESULTS: After 26±5years, 6% (1/18) and 44% (8/18) suffered clinic hypertension and prehypertension, respectively. On 24-h blood pressure monitoring, 15% (3/20) and 20% (4/20) had hypertension and prehypertension, respectively. Coarctation patients had elevated muscle sympathetic nerve activity compared with controls (49.6±24.9 vs. 29.9±14.0 bursts/100 heartbeats, p=0.02), dampened sympathetic baroreflex function (-2.2±2.1 vs. -7.0±5.6 bursts/100heartbeats·mm·Hg-1, p=0.007), normal cardiac baroreflex function (41.9±30.4 vs. 35.7±21.1ms·mm·Hg-1, p=0.6), endothelial dysfunction (pulse amplitude tonometry ratio: 0.39±0.32 vs. 0.81±0.50, p=0.004), and increased ambulatory arterial stiffness index (0.46±0.15 vs. 0.29±0.17, p=0.008).
CONCLUSION: After coarctation repair patients have increased muscle sympathetic nerve activity, dampened sympathetic baroreflex response, endothelial dysfunction, and increased ambulatory arterial stiffness index, all of which may contribute to the development of late hypertension.

PMID: 28545853 [PubMed - indexed for MEDLINE]

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Management of Marfan Syndrome during pregnancy: A real world experience from a Joint Cardiac Obstetric Service.

Int J Cardiol. 2017 Sep 15;243:180-184

Authors: Lim JCE, Cauldwell M, Patel RR, Uebing A, Curry RA, Johnson MR, Gatzoulis MA, Swan L

BACKGROUND: Pregnancy in Marfan Syndrome (MFS) is associated with increased maternal risk of cardiovascular events. Given the maternal and genetic risks, pre-conception counselling is essential to facilitate informed choices. Multidisciplinary antenatal care with regular imaging is mandatory and best delivered through a Joint Cardiac Obstetric Service (JCOS). The aim of this study was to compare the care delivered in a JCOS against recognised international standards (European Society of Cardiology (ESC)).
METHODS: Pregnancies in women with MFS from 2005 to 2015 were identified from our institutional database. Patient records were reviewed and practice assessed against pre-determined standards based on ESC guidelines.
RESULTS: There were 23 pregnancies in 15 women with MFS. 13/23 (57%) occurred in women with aortic dilatation at baseline. There were 3 important maternal cardiac events (type A dissection; deterioration in left ventricular function; significant left ventricular and progressive aortic dilatation). Four women did not have access to expert pre-conception counselling. These women were all referred to the JCOS late in established pregnancy. Imaging was often delayed and only 7/23 cases (30%) met the standard for minimum frequency of echocardiographic surveillance. Only 12/23 (52%) had pre-conception imaging of the whole aorta with CT/MRI. Distal aortic dilatation was identified in 7/23 cases but none of these underwent further MRI evaluation during pregnancy.
CONCLUSION: Despite having a dedicated JCOS, our data show that facilitating complete obstetric and cardiac care for this group remains challenging. Education of local care providers and timely referral for expert pre-conception counselling in a JCOS are key.

PMID: 28606654 [PubMed - indexed for MEDLINE]