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Misclassification of bicuspid aortic valves is common and varies by imaging modality and patient characteristics.

Echocardiography. 2019 Mar 04;:

Authors: Cramer PM, Prakash SK

BACKGROUND: Bicuspid aortic valve (BAV) is the most prevalent adult congenital heart defect. BAV causes lifelong progressive disease that can be prevented by early diagnosis and long-term surveillance, but may be compromised by misclassification of valve morphology.
METHODS: The study population was derived from the UTHealth Bicuspid Aortic Valve Registry, which includes serial images on more than 200 participants over a mean follow-up interval of 2.8 years. We abstracted descriptions of aortic valve morphology from transthoracic or transesophageal echocardiography, computed tomography angiography, and magnetic resonance angiography reports. We used chi-square and t tests to determine associations between reported valve morphologies (definitely bicuspid, possibly bicuspid, tricuspid, or uncertain) and clinical characteristics and assessed image quality using a validated tool.
RESULTS: About 40% of participants were misclassified in at least one imaging report. The mean interval between misclassification and correct diagnosis was 22 months. TEE, MR and CT were more sensitive than TTE and successfully reclassified 20% of participants, but were only used in 14% of patients. Misclassification was associated with age, the extent of valve calcification and image quality, but was not significantly associated with aortic regurgitation, gender, or cusp configuration.
CONCLUSION: Misclassification of BAV is prevalent, frequently leads to delayed diagnosis, and is more likely to occur in the most severely affected cases. TEE, CT and MR may increase diagnostic accuracy in up to half of BAV cases but are underutilized. Additional studies are needed to determine if misclassification of BAV patients leads to increased long-term morbidity and mortality.

PMID: 30834578 [PubMed - as supplied by publisher]

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Predictors of extracorporeal membrane oxygenation support after surgery for adult congenital heart disease in children's hospitals.

Congenit Heart Dis. 2019 Mar 05;:

Authors: Dolgner SJ, Krieger EV, Wilkes J, Bratton SL, Thiagarajan RR, Barrett CS, Chan T

OBJECTIVE: Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children's hospitals.
DESIGN: All adults (≥18 years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included. Patients were classified into three groups: ECMO-free survival, requiring ECMO, and DWE. Univariate analyses were performed, and multinomial logistic regression models were constructed examining ECMO and DWE as independent outcomes.
SETTING: Tertiary care children's hospitals.
RESULTS: A total of 4665 adult patients underwent ACHD surgery in 39 children's hospitals with 51 (1.1%) patients requiring ECMO and 64 (1.4%) patients experiencing DWE. Of the 51 ECMO patients, 34 (67%) died. Increasing patient age, surgical complexity, diagnosis of single ventricle heart disease, preoperative hospitalization, and the presence of noncardiac complex chronic conditions (CCC) were risk factors for both outcomes. Additionally, low and medium hospital ACHD surgical volume was associated with an increased risk of DWE in comparison with ECMO.
CONCLUSIONS: There are overlapping but separate risk factors for ECMO support and DWE among adults undergoing congenital heart surgery in pediatric hospitals.

PMID: 30835967 [PubMed - as supplied by publisher]

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Authors' Reply.

J Am Soc Echocardiogr. 2017 10;30(10):1041

Authors: Baumgartner H, Hung J

PMID: 28864151 [PubMed - indexed for MEDLINE]

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Cardiac Magnetic Resonance Evaluation of Pulmonary Transit Time and Blood Volume in Adult Congenital Heart disease.

J Magn Reson Imaging. 2019 Mar 05;:

Authors: Ait Ali L, Aquaro GD, Peritore G, Ricci F, De Marchi D, Emdin M, Passino C, Festa P

BACKGROUND: Management of adults with repaired congenital heart disease (CHD) is still challenging. Heart failure secondary to residual anatomical sequels or arrhythmic events is not rare in this population. MRI has emerged as an accurate tool to quantify pulmonary transit time (PTT) of intravenous contrast agents and pulmonary blood volume (PBV).
PURPOSE: To determine the relationship between PTT, and conventional indexes of ventricular dysfunction and heart failure in a cohort of adults with CHD and to assess its association with adverse outcomes.
STUDY TYPE: Retrospective.
SUBJECTS: 89 adult CHD patients (56 males, age 34 ± 11 years) and 14 age- and sex-matched healthy subjects.
FIELD STRENGTH/SEQUENCE: First-pass perfusion and standard sequences for ventricular volumes and function and flow analysis at 1.5T.
ASSESSMENT: PTT was calculated as the time required for a bolus of contrast agent to pass from the right ventricle to the left atrium, expressed both in seconds (PTTS) and number of heartbeats (PTTB). The pulmonary blood volume index (PBVI) was measured by the product of PTTB and the pulmonary artery stroke volumes.
STATISTICAL TESTS: Student's independent t-test analysis of variance (ANOVA) and Mann-Whitney nonparametric; Pearson's or Spearman's correlation; Kaplan-Meier method.
RESULTS: PTTS and PTTB were significantly higher in patients than in controls (7.6 ± 3 vs. 5.6 ± 1.2 sec, P = 0.01 and 8 ± 3 vs. 6 ± 1 bpm, P = 0.01, respectively). PTTS showed negative correlation with left ventricle ejection fraction (LVEF) and cardiac index (CI) (r = -0.3, P = 0.004, and r = -0.4, P < 0.001, respectively) as well as with left ventricle and atrial volumes. By Kaplan-Meier survival analysis, PTTB >8 bpm was associated with significant increased risk of adverse outcome at mid-term follow-up. Moreover, patients with both increased PTTB and PBV have higher amino-terminal portion of the prohormone brain natriuretic peptide (NT-proBNP) and lower LVEF.
DATA CONCLUSION: PTT is prolonged in adult CHD in comparison with healthy subjects, likely reflecting reduced CI and ventricular dysfunction.
LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2019.

PMID: 30838716 [PubMed - as supplied by publisher]

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Porcine Versus Pericardial Pulmonary Valve Replacement in Adults With Prior Congenital Cardiac Surgery: Midterm Outcomes.

World J Pediatr Congenit Heart Surg. 2019 Mar;10(2):197-205

Authors: Yuen CS, Lee KFL, Bhatia I, Yam N, Rocha BA, Yung TC, Chow PC, Au WT

BACKGROUND:: Postcongenital heart surgery pulmonary regurgitation requires subsequent pulmonary valve replacement. We sought to compare the outcomes of pulmonary valve replacement after using bioprosthetic valves, porcine versus pericardial bioprosthesis.
METHOD:: Retrospective single-center study of consecutive pulmonary valve replacement in patients with pulmonary regurgitation following initial congenital cardiac surgery. From 2004 to 2016, 82 adult patients (53 males, 29 females) underwent pulmonary valve replacement at a mean age of 28.7 ± 8 years (range 18-52 years) with a mean time to pulmonary valve replacement of 24 ± 7 years (range 13-43 years). Porcine bioprosthetic valves (group 1, n = 32) and pericardial valves (group 2, n = 50) were used. Cardiac magnetic resonance imaging was performed (n = 54) at a mean of 18 ± 13 months before and 24 ± 21 months after pulmonary valve replacement.
RESULTS:: No significant difference was seen between the groups except that the mean follow-up was longer for group 1 (5.02 ± 2.06 vs 4.08 ± 3.21 years). In-hospital mortality was 1.1%. Follow-up completeness was 100% with no late death. Mean right ventricular end-systolic and end-diastolic volumes reduced significantly in both the groups ( P < .001), whereas right ventricular ejection fraction remained unchanged (group 1, P = .129; group 2, P = .675) . Only the left ventricular end-diastolic volume increased in both the groups, but the increase was significant for group 2 only (group 1, P = .070; group 2, P = .015), whereas the left ventricular end-systolic and ejection fraction remained unchanged in both the groups. There was no reoperation for pulmonary valve replacement. Freedom from intervention was 93.8% (group 1) and 100% (group 2) at eight years after pulmonary valve replacement ( P = .407).
CONCLUSION:: Midterm outcomes of pulmonary valve replacement in our adult cohort were satisfactory. Both types of bioprosthetic valves performed comparably for eight years and were a good option in adults.

PMID: 30841830 [PubMed - in process]

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Total Anomalous Pulmonary Venous Connection Beyond the First Decade of Life.

World J Pediatr Congenit Heart Surg. 2019 Mar;10(2):185-191

Authors: Talwar S, Arora Y, Gupta SK, Kothari SS, Ramakrishnan S, Saxena A, Choudhary SK

OBJECTIVE:: We report our experience with surgery for total anomalous pulmonary venous connection (TAPVC) beyond first decade of life.
METHODS:: Between January 1987 and July 2017, 98 patients ≥ten years underwent TAPVC repair. Their detailed case-records were analyzed.
RESULTS:: Mean age was 19.05 (± 12.8; median 18) years. Anatomic subtypes were supracardiac (n = 62), cardiac (n = 20), and mixed (n = 16). An atrial septal defect (ASD) was present in all. Severe tricuspid valve regurgitation was present in four patients and severe rheumatic mitral regurgitation was present in one. On preoperative cardiac catheterization, mean pulmonary artery pressure was 67 ± 15.6 mm Hg (median 58; range 37-96). Mean pulmonary vascular resistance was 5.6 ± 3.9 Woods units·m2 (median 4.7, range 2.9-11.8). Twenty-five patients had moderate and eight had severe pulmonary arterial hypertension. Right ventricular dysfunction was present in eight patients. At repair, a small ASD was left open in 87 patients while in 11 patients, the ASD was closed using a unidirectional valved patch. Follow-up was available for 90 (92%) patients. There were no late deaths over a mean follow-up of 163 ± 103.98 months (median 163, range 1-362). Eighty-two patients were in NYHA class I and eight were in class II. Right ventricular function normalized in 82 patients while 80 patients had reduction in pulmonary artery pressure. Event-free survival was 94.2% at 5 years, 92.3% at 10 years, and 90% at 20 and 30 years.
CONCLUSIONS:: Outcomes of surgical repair for TAPVC beyond first decade of life are satisfactory. Close follow-up, however, is necessary for possible persistence of pulmonary arterial hypertension.

PMID: 30841831 [PubMed - in process]

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Familial Aggregation and Heritability of Congenital Heart Defects.

Circ J. 2017 12 25;82(1):232-238

Authors: Kuo CF, Lin YS, Chang SH, Chou IJ, Luo SF, See LC, Yu KH, Huang LS, Chu PH

BACKGROUND: Congenital heart defects (CHD) are known to cluster within families, but existing evidence varies for the estimates of familial relative risk (RR). We aimed to examine familial aggregation and heritability of CHD in the general population of Taiwan.Methods and Results:We conducted a population-based family study using the Taiwan National Health Insurance (NHI) research database. Individuals with affected first-degree (n=295,636) or second-degree (n=73,985) relatives were identified from all NHI beneficiaries (n=23,422,955) registered in 2012. Diagnoses of CHD for all study subjects were ascertained between January 1, 1996 and December 31, 2012. Having a twin, a first-degree relative and an affected second-degree relative were associated with an adjusted RR of 12.03 (11.59-12.49), 4.91 (4.85-4.97) and 1.21 (1.14-1.28) for CHD, respectively. Individuals with 1 affected first-degree relative had a RR of 4.78 (4.72-4.84), and those with ≥2 had an RR of 7.10 (6.77-7.45) for CHD. The estimated accountability for phenotypic variance of CHD was 37.3% for familial transmission and 62.8% for non-shared environmental factors.
CONCLUSIONS: Our results indicated that CHD tend to cluster within families, and approximately one-third of phenotypic variance was explained by familial factors.

PMID: 28824028 [PubMed - indexed for MEDLINE]

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Echocardiographic evaluation of prevalence of pulmonary hypertension in β-thalassemia major: A cross sectional study.

Pediatr Hematol Oncol. 2018 Aug - Sep;35(5-6):322-330

Authors: Rashidi F, Sate H, Mohammadi A, Koohi A, Nejati B, Naybzadeh A

INTRODUCTION: Pulmonary hypertension is a common complication associated with thalassemia syndromes and it may play an important role in the pathogenesis of right ventricle failure. The true prevalence of pulmonary hypertension in patients with thalassemia major remains unclear and has been reported to be between 2 and 79%.
MATERIALS AND METHODS: In total, 70 patients with thalassemia major were initially examined. Patients with valvular left heart disease, congenital heart diseases such as atrial septal defect (ASD) and ventricular septal defect (VSD), left heart failure, and chronic embolism were excluded. All patients with thalassemia major underwent echocardiography. Based on tricuspid regurgitation velocity (TRV), the patients were divided into the following three groups: low, medium, and high risk of pulmonary hypertension.
RESULTS: The mean age of the subjects was 24 y; 60.6% of the subjects were males and 39.4% of the subjects were females. Overall, three (4.5%) subjects were considered at a high risk of pulmonary hypertension. The mean hemoglobin level in the patients with a high probability of pulmonary hypertension was 8.2 g/dL and that in the patients with a low or medium probability of pulmonary hypertension was 9.1 g/dL. No significant difference was observed between the groups (p = .059).
CONCLUSION: This study showed that, based on new echocardiography criteria, the prevalence of pulmonary hypertension secondary to β-thalassemia was 4.5% and there was no correlation between TRV and the number of received blood units or disease duration.

PMID: 30468099 [PubMed - indexed for MEDLINE]

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Biological versus mechanical heart valve prosthesis during pregnancy in women with congenital heart disease.

Int J Cardiol. 2018 Oct 01;268:106-112

Authors: Lameijer H, van Slooten YJ, Jongbloed MRM, Oudijk MA, Kampman MAM, van Dijk AP, Post MC, Mulder BJ, Sollie KM, van Veldhuisen DJ, Ebels T, van Melle JP, Pieper PG

BACKGROUND: We evaluate pregnancy outcome and anticoagulation regimes in women with mechanical and biological prosthetic heart valves (PHV) for congenital heart disease.
METHODS: Retrospective multicenter cohort studying pregnancy outcomes in an existing cohort of patients with PHV.
RESULTS: 52 women had 102 pregnancies of which 78 pregnancies (46 women) ≥20 weeks duration (59 biological, 19 mechanical PHV). Miscarriages (n = 19, ≤20 weeks) occurred more frequently in women using anticoagulation (P < .05). During 42% of pregnancies of women with mechanical PHV a combined low molecular weight heparin (LMWH) vitamin-K-antagonist anticoagulation regime was used (n = 8). Overall, cardiovascular, obstetric and fetal/neonatal complications occurred in 17% (n = 13), 68% (n = 42) and 42% (n = 27) of the pregnancies. Women with mechanical PHV had significantly higher cardiovascular (12% vs 32%, P < .05), obstetric (59% vs 85%, P = .02) and fetal/neonatal (34% vs 61%, P < .05) complication rates than women with biological PHV. This was related to PHV thrombosis (n = 3, P < .02), post-partum hemorrhage (P < .02), cesarean section (P < .02), low birth weight and small for gestational age (both P < .05). PHV thrombosis occurred in 3 pregnancies, including 2/5 pregnancies with pulmonary mechanical PHV. PHV thrombosis was related to necessary cessation of anticoagulation therapy or insufficient monitoring of LMWH. Other cardiovascular complications occurred equally frequent in both groups.
CONCLUSION: Complications occur more often in pregnancies of women with a mechanical PHV than in women with a biological PHV, mainly caused by PHV thrombosis and bleeding complications. Meticulous monitoring of anticoagulation in pregnant women is necessary. Women with a pulmonary mechanical PHV are at high risk of complications.

PMID: 29848449 [PubMed - indexed for MEDLINE]

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Role of genetic heart disease in sentinel sudden cardiac arrest survivors across the age spectrum.

Int J Cardiol. 2018 Nov 01;270:214-220

Authors: Giudicessi JR, Ackerman MJ

BACKGROUND: Sudden cardiac arrest (SCA) may be the sentinel expression of a sudden cardiac death-predisposing genetic heart disease (GHD). Although shown to underlie many unexplained SCAs in the young, the contribution of GHDs to sentinel SCA has never been quantified across the age spectrum. Thus, we sought to determine the contribution of GHDs in single-center referral cohort of non-ischemic SCA survivors.
METHODS AND RESULTS: Retrospective analysis of 3037 patients was used to identify all individuals who experienced a sentinel event of SCA. Following exclusion of patients with ischemic or complex congenital heart disease, cases were classified by clinical diagnoses. Overall, 180 (5.9%) referral patients experienced a sentinel SCA (average age at SCA 28 ± 15 years, 99 females). An etiology was identified in 113/180 patients (62.8%) including channelopathies in 26.7%, arrhythmogenic bileaflet mitral valve prolapse in 10.6%, cardiomyopathies in 9.4%, other etiologies in 6.7%, acquired long QT syndrome in 6.7%, and multiple disorders in 2.8%. The remaining 67/180 (37.2%) cases were classified as idiopathic ventricular fibrillation (IVF). Interestingly, the contribution of GHDs declined precipitously after the first decade of life [90.0% (age 0-9; n = 20), 58.7% (age 10-19; n = 46), 28.1% (age 20-29; n = 32), 23.8% (age 30-39; n = 42), 16.7% (age 40-49; n = 24), and 12.5% (age 50+; n = 16)].
CONCLUSIONS: Within a referral population enriched for GHDs, the ability of a comprehensive cardiac evaluation, including genetic testing, to elucidate a root cause in non-ischemic SCA survivors declined with age. Although rare, GHDs can underlie SCA into adulthood and merit consideration across the age spectrum.

PMID: 29884292 [PubMed - indexed for MEDLINE]