Univentricular hearts in Denmark 1977 to 2009: Incidence and survival

Idorn, Lars; Olsen, Morten; Jensen, Annette Schophuus; Juul, Klaus; Reimers, Jørgen; Sørensen, Keld E.; Johnsen, Søren P; Søndergaard, Lars

doi:10.1016/j.ijcard.2012.03.182

Cited by 56 publications

(62 citation statements)

References 22 publications

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“…Using the same classification, the mean corrected incidence rate of univentricular heart (live births and terminated pregnancies) in the Danish Register of Congenital Heart Disease was 0.39 per 1000 live births per year [11], very similar to the combined unadjusted incidence of most common univentricular heart lesions in the study by Samanek and Voriskova [12]. Comparatively, the corrected incidence in our study was 0.69 per 1000 live births per year.…”

Section: Discussionsupporting

confidence: 85%

“…As in other cohorts [11,13,14], most of the deaths in the current study occurred in the first few months of life, and after the first year of life, the survival curves plateaued, which can be explained by the survival of children with a more favorable anatomy and without extra major cardiac defects. It also indicates the critical importance of meticulous intensive care in the neonatal period and timely interventions.…”

Section: Discussionsupporting

confidence: 78%

“…It is likely that this difference is due to the low total numbers and the rarity of the disease [1]. The percentage of terminated pregnancies we observed in our cohort was very high at 76.6%; higher than the 66.9% reported in the UK in 1999 [10], and even more so than the 34.6% reported in Denmark from 2000 until 2009 [11]. A hypothetical explanation for the higher termination rate is that since Latvia joined the European Union, many of its citizens of reproductive age work and give birth abroad, but return home for medical treatment.…”

Section: Discussionmentioning

confidence: 68%

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Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015

et al. 2018

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Background and Objectives: A functionally univentricular heart is the term used to describe congenital heart defects where it is impossible to restore two pumping chambers. These lesions are associated with high mortality, morbidity, and medical resource utilization. The aim of this study was to review incidence and outcomes of patients with a functionally univentricular heart at the only pediatric cardiac surgery center in Latvia. Methods: We performed a retrospective review of medical records of (i) all children with a functionally univentricular heart treated at the Clinic of Pediatric Cardiology and Cardiac Surgery, and (ii) all prenatally diagnosed cases of univentricular heart at Children’s Clinical University Hospital in Latvia. We reviewed data regarding children born from January 1, 2007, to December 31, 2015. The children’s cardiac anatomy and interventions were categorized in accordance with the International Pediatric and Congenital Cardiac Code (v3.3). Results: During the study period, 49 patients with a functionally univentricular heart were admitted to Children’s Clinical University Hospital with a corrected incidence of 0.69 per 1000 live births per year. There were 26 patients that had a hypoplastic left ventricle, and 22 patients that had a hypoplastic right ventricle, while one patient had an indeterminate ventricle. Thirty (61.2%) patients had died by the end of data collection. Twenty-one of the 30 deaths occurred before or immediately after stage I surgical palliation. Cumulative neonatal and 5-year survival of patients with a hypoplastic right ventricle was 81.8% and 63.6%, respectively; for patients with hypoplastic left ventricle—46.2% and 17.3%, respectively. Discussion: This is the first mid-term outcome study of patients with a univentricular heart in Latvia. The high mortality reflects the challenges of a small-volume, developing congenital cardiac surgery center. Data from this study will be used as a baseline for quality improvement.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 68%

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Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015

et al. 2018

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“…2 Although the surgical techniques have evolved, 3,4 the procedure remains palliative with current 10-year survival rates reaching 90%. 5,6 The prevalence of Fontan patients has consequently increased significantly over the past decades, 7 and clinicians are now frequently caring for patients who suffer from a number of late comorbidities, including arrhythmias and exercise intolerance. These problems will continue to rise markedly.…”

Section: Introduction Of the Fontan Operation In 1971mentioning

confidence: 99%

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

et al. 2014

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Background— The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. Methods and Results— Seventy-five adolescents and adults were randomized 1:1 to 14 weeks of treatment with bosentan or placebo. Cardiopulmonary exercise test, functional class, blood samples, and quality-of-life questionnaires were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 mL·kg −1 ·min −1 (from 28.7 to 30.7 mL·kg −1 ·min −1 ) in the bosentan group compared with 0.6 mL·kg −1 ·min −1 (from 28.4 to 29.0 mL·kg −1 ·min −1 ) in the placebo group ( P =0.02). Cardiopulmonary exercise test time increased by 0.48 minute (from 6.79 to 7.27 minutes) versus 0.08 minute (from 6.94 to 7.02 minutes; P =0.04). Nine bosentan-treated patients improved 1 functional class, whereas none improved in the placebo group ( P =0.0085). Side effects were mild and occurred equally in both groups. No serious adverse effects were seen, and no patients had liver enzyme levels above the 3-fold upper limit. Conclusions— Bosentan improves exercise capacity, exercise time, and functional class in Fontan patients without serious adverse events or hepatotoxicity. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT01292551.

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“…The reported incidence of TE in these patients varies from 3% to 25%, depending on study design, imaging technique, and follow-up period duration. Studies with longer follow-up periods and more sensitive imaging studies suggest an incidence of at least 20%, of which the mortality rate is 25% 5–9. Asymptomatic pulmonary emboli have been detected in 16% of TCPC patients.…”

Section: Discussionmentioning

confidence: 99%

Major stroke in a 19-year-old patient with a univentricular heart

Riemann¹,

Idorn²,

Wagner³

et al. 2013

IJGM

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Patients with univentricular heart malformations are at increased risk of suffering from thromboembolic events. We present a case of a 19-year-old woman born with a univentricular heart who suffered a major stroke while being treated with only salicylic acid. At least 20% of patients with univentricular hearts have been reported to experience thromboembolic events, of which 25% are fatal. Despite the high incidence of thromboembolic events, no consensus has been reached regarding the role of long-term anti-thrombotic treatment in this group of patients. This lack of consensus warrants future studies that compare the different therapeutic strategies.

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Univentricular hearts in Denmark 1977 to 2009: Incidence and survival

Cited by 56 publications

References 22 publications

Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015

Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation

Major stroke in a 19-year-old patient with a univentricular heart

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