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Pregnancy in Women With a Fontan Circulation: A Systematic Review of the Literature.

Circ Cardiovasc Qual Outcomes. 2018 05;11(5):e004575

Authors: Garcia Ropero A, Baskar S, Roos Hesselink JW, Girnius A, Zentner D, Swan L, Ladouceur M, Brown N, Veldtman GR

BACKGROUND: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainly of anecdotal experience and small series. This article seeks to review the published literature and identify lessons learnt from this collective experience.
METHODS AND RESULTS: We conducted a systematic review to evaluate maternal and fetal outcomes of pregnancy in women with a Fontan circulation. Among 1150 studies that were screened, 6 studies had sufficient longitudinal data points to qualify for meaningful inclusion, yielding 255 pregnancies in 133 women after Fontan procedure resulting in 115 live births (45%; including reports from 1986 to 2015). There was a total of 137 pregnancy losses (69%), with 115 miscarriages (45%), 19 elective terminations of pregnancy (7%), 2 stillbirths (1%), and 1 ectopic pregnancy (1%).The most common cardiovascular adverse events were supraventricular arrhythmia affecting 8.4% (range, 3%-37%) and heart failure affecting 3.9% (range, 3%-11%) of pregnancies. These complications were successfully managed with conventional approaches. No maternal deaths were reported. Postpartum hemorrhage was the predominant obstetric complication affecting 14% of the patients. Most patients were on antiplatelet agents (27%) or anticoagulants (50%) whereas only a minority (11%) were on neither. Among the 115 live births, 68 were premature (59%), 17 were small for gestational age (20%), and neonatal death occurred in 6 patients (5%).
CONCLUSIONS: The most commonly reported cardiovascular complications in patients with Fontan physiology-associated pregnancy were arrhythmia and heart failure. Miscarriages were highly prevalent as was prematurity and intrauterine growth restriction. Postpartum hemorrhage seems to be the most common obstetric complication. Large-scale data sets are needed to confirm these early observations and address the late sequelae of pregnancy in women with a Fontan circulation.

PMID: 29752389 [PubMed - indexed for MEDLINE]

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Genetic variation in folate metabolism is associated with the risk of conotruncal heart defects in a Chinese population.

BMC Pediatr. 2018 08 30;18(1):287

Authors: Wang X, Wei H, Tian Y, Wu Y, Luo L

BACKGROUND: Conotruncal heart defects (CTDs) are a subgroup of congenital heart defects that are considered to be the most common type of birth defect worldwide. Genetic disturbances in folate metabolism may increase the risk of CTDs.
METHODS: We evaluated five single-nucleotide polymorphisms (SNPs) in genes related to folic acid metabolism: methylenetetrahydrofolate reductase (MTHFR C677T and A1298C), solute carrier family 19, member 1 (SLC19A1 G80A), methionine synthase (MTR A2576G), and methionine synthase reductase (MTRR A66G), as risk factors for CTDs including various types of malformation, in a total of 193 mothers with CTD-affected offspring and 234 healthy controls in a Chinese population.
RESULTS: Logistic regression analyses revealed that subjects carrying the TT genotype of MTHFR C677T, the C allele of MTHFR A1298C, and the AA genotype of SLC19A1 G80A had significant 2.47-fold (TT vs. CC, OR [95% CI] = 2.47 [1.42-4.32], p = 0.009), 2.05-2.20-fold (AC vs. AA, 2.05 [1.28-3.21], p = 0.0023; CC vs AA, 2.20 [1.38-3.58], p = 0.0011), and 1.68-fold (AA vs. GG, 1.68 [1.02-2.70], p = 0.0371) increased risk of CTDs, respectively. Subjects carrying both variant genotypes of MTHFR A1298C and SLC19A1 G80A had a higher (3.23 [1.71-6.02], p = 0.0002) increased risk for CTDs. Moreover, the MTHFR C677T, MTHFR A1298C, and MTRR A66G polymorphisms were found to be significantly associated with the risk of certain subtypes of CTD.
CONCLUSIONS: Our data suggest that maternal folate-related SNPs might be associated with the risk of CTDs in offspring.

PMID: 30165839 [PubMed - indexed for MEDLINE]

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The role of long-term mechanical circulatory support in the treatment of end-stage heart failure.

Kardiol Pol. 2019;77(3):331-340

Authors: Paluszkiewicz L, Kukulski T, Zembala M, Gummert J, Morshuis M

Heart failure is a clinical syndrome of multifactorial aetiology with typical symptoms and diverse prevalence depending on the world region, reaching more than 10% in the population over 70 years of age. The prognosis, in spite of a dynamic improve- ment in medical therapy, remains poor. The only treatment for these patients is heart transplantation, however, its availability is highly limited because of the shortage of donor organs. Mechanical circulatory support can offer an alternative treatment for this patient cohort. In this review the authors discuss the present indications for, as well as results and complications of different types of long-term mechanical circulatory support. The long-term survival in patients receiving this therapy, in spite of many complication, is much better than in those receiving medical treatment. The use of mechanical circulatory support is an established treatment option for many patients with end-stage heart failure. The most important issue for the cardiologist is to identify patients eligible for this therapy in order to give them a chance for a longer life and better quality of life.

PMID: 30915780 [PubMed - indexed for MEDLINE]

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Hypertrophic cardiomyopathy: New hope for an old disease.

Nursing. 2019 Sep;49(9):24-31

Authors: Larkin G, Bellomo T, Caze L

Hypertrophic cardiomyopathy (HCM), a common congenital heart disease, is the leading cause of sudden cardiac death in adolescents, young adults, and athletes. Older adults with HCM are less likely to experience sudden cardiac death, but their quality of life can be impaired. This article discusses diagnostic criteria, treatments, and nursing interventions, including patient teaching, for adults with HCM.

PMID: 31365456 [PubMed - indexed for MEDLINE]

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Staged transcatheter closure for atrial septal defect and patent ductus arteriosus: a case report.

Eur Heart J Case Rep. 2019 Jun 01;3(2):

Authors: Soma Y, Shiraishi Y, Kanazawa H, Fukuda K

BACKGROUND: Atrial septal defect (ASD) and patent ductus arteriosus (PDA) are both common congenital heart diseases, but the combination of these two cardiac defects is extremely rare, and the therapeutic strategy is controversial.
CASE SUMMARY: We treated an adult patient with combined ASD and PDA, and safely attained a successful outcome with two-stage transcatheter closure, which is PDA closure preceding ASD closure, to prevent serious complications.
DISCUSSION: Transcatheter closure of one of the defects is now widely accepted as an alternative to surgical closure. In addition, adults with both ASD and PDA are better suited for transcatheter closure than surgical closure. One of the reasons is the difficulty to ligate the ductus arteriosus of an adult patient due to its friability and calcification. Meanwhile, simultaneous combined transcatheter closure of ASD and PDA can result in serious complications, such as thrombocytopenia and haemolysis, whose mechanism is considered to be the destruction of platelets and red blood cells by the residual shunt through implanted devices. Additionally, antiplatelet therapy that prevents device-related thrombus formation after ASD closure can possibly exacerbate thrombocytopenia and haemolysis. Therefore, the staged strategy is reasonable from the perspectives of antiplatelet therapy and haemodynamic changes.

PMID: 31449609 [PubMed]

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A case report: stent implantation to treat coarctation of persistent 5th aortic arch associated with interrupted 4th aortic arch.

Eur Heart J Case Rep. 2019 Jun 01;3(2):

Authors: Kato A, Ohashi N, Nishikawa H

BACKGROUND: Persistent 5th aortic arch is a rare cardiac anomaly that is usually surgically corrected during infancy or early childhood if it is associated with coarctation of the aorta. Here, we report an adult with coarctation of the 5th aortic arch who was successfully treated by stent implantation.
CASE SUMMARY: An asymptomatic 32-year-old woman presented with hypertension and a significant arm-leg difference in pressure. On suspicion of coarctation of the aorta, a chest computed tomography was performed, leading to a diagnosis of an interrupted 4th aortic arch with coarctation of a persistent 5th aortic arch. Percutaneous catheter intervention using a PALMAZ large stent dilated to 12 mm resulted in a minimal peak-to-peak pressure gradient. The patient was discharged home after a 2-day monitoring without hypertension and arm-leg blood pressure difference. She remained normotensive with a patent aortic arch on echocardiography performed 10 months after treatment.
DISCUSSION: As for simple coarctation of the aorta, stent implantation was feasible and effective in an adult patient with coarctation of the 5th aortic arch.

PMID: 31449629 [PubMed]

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Successful treatment of cardiac haemangiomas with oral propranolol: a case series of two patients.

Eur Heart J Case Rep. 2019 Jun 01;3(2):

Authors: Polymerou I, Ojala T, Bonou P, Martelius L, Tzifa A

BACKGROUND: Cardiac haemangiomas are extremely rare tumours with equivocal surgical outcomes. Haemangiomas appearing on other sites of the body have been successfully treated with oral propranolol. To the best of our knowledge, such treatment has not been tried to date for cardiac location of haemangiomas.
CASE SUMMARY: We report two cases of neonatal cardiac haemangiomas, and we describe their presentation and characteristics, as well as how these were successfully treated with oral propranolol, with complete regression of the tumours within the 1st year of life.
DISCUSSION: Despite the rarity of cardiac haemangiomas, their presentation and complications could be dramatic with side-effects spanning from intracardiac space occupying phenomena to Kasabach-Merritt syndrome. Propranolol therapy, having been established for long now in the treatment of skin haemangiomas, should also be considered in cases of cardiac haemangiomas, particularly in the neonatal and infantile population.

PMID: 31449646 [PubMed]

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Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study.

Int J Cardiol. 2019 Feb 01;276:74-80

Authors: Ohuchi H, Inai K, Nakamura M, Park IS, Watanabe M, Hiroshi O, Kim KS, Sakazaki H, Waki K, Yamagishi H, Yamamura K, Kuraishi K, Miura M, Nakai M, Nishimura K, Niwa K, JSACHD Fontan Investigators

BACKGROUND: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear.
PURPOSE: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants.
METHOD AND RESULTS: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ± 1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years.
CONCLUSIONS: Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.

PMID: 30201381 [PubMed - indexed for MEDLINE]

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Do we differ in terms of indications and demographics in cardiac resynchronisation recipients in Poland? Insights from the European CRT Survey II Registry.

Kardiol Pol. 2019;77(1):40-46

Authors: Łasocha D, Sterliński M, Tajstra M, Josiak K, Zając D, Tłuczek S, Sokal A, Januszkiewicz ŁJ, Galar B, Sznajder R, Kaźmierczak J, Szumowski Ł, Normand C, Linde C, Dickstein K

BACKGROUND: Multiple randomised clinical trials have proven that cardiac resynchronisation therapy (CRT) reduces morbidity and mortality in appropriately selected patients with congestive heart failure and is recommended for such patients as per the European Society of Cardiology guidelines.
AIM: In this paper we compare the indications and demographics in cardiac resynchronisation recipients in Poland and other European countries.
METHODS: In 2015 and 2016, physicians from 42 European countries participated in the second edition of the European Cardiac Resynchronisation Therapy Survey. For 14 months, 288 implanting centres gathered data regarding demography, indications, implanting methods, and guidance compatibility from 11,088 patients receiving CRT.
RESULTS: The survey revealed that a vast group of patients were eligible for CRT implantation (although some of them with rela-tively weak guidance recommendations) and showed essential variety in clinical practice when national data were benchmarked.
CONCLUSIONS: The population of CRT recipients in Poland and other European countries did not differ in terms of demographic and clinical characteristics. In most cases, indications for CRT were in accordance with the guidelines; however some devices were implanted in patients beyond the guideline recommendations. For these procedures, the decision regarding CRT im-plantation relies mainly on the physicians' experience.

PMID: 30406939 [PubMed - indexed for MEDLINE]

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Sudden cardiac death in a young adult man due to spontaneous coronary artery dissection.

Malays J Pathol. 2019 Apr;41(1):51-54

Authors: Subramaniam K, Siew SF, Mahmood MS

Spontaneous coronary artery dissection is a rare event and commonly associated with pregnancy and female gender. This condition can reduce or completely obstruct the blood flow to the heart, causing a myocardial ischaemia, abnormalities in heart rhythm or sudden death. We present a case of a 28-year-old Indian male with no previous medical illness who complained sudden onset of chest pain prior to his death. Autopsy revealed a left anterior descending coronary artery dissection associated with plaque rupture. The anterior wall of left ventricle showed contraction band necrosis. There was also atheroma present in the right coronary artery which was insignificant. Histologically, dissection was associated with atherosclerosis. There was no evidence of vasculitis. The cause of death was given as coronary artery dissection due to coronary artery atherosclerosis.

PMID: 31025638 [PubMed - indexed for MEDLINE]