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10 pages, 385 KiB

Open AccessArticle

Prevalence and Treatment Outcomes of Arrhythmias in Patients with Single Ventricle Physiology over the Age of 40 Years

by Claudia Pujol, Gabriele Hessling, Marta Telishevska, Sandra Schiele, Isabel Deisenhofer, Peter Ewert and Oktay Tutarel

J. Clin. Med. 2022, 11(21), 6568; https://doi.org/10.3390/jcm11216568 - 5 Nov 2022

Cited by 5 | Viewed by 1900

Abstract

Background: Arrhythmias are a well known complication in patients with single ventricle physiology (SVP). However, there is still a lack of data regarding arrhythmias in older patients. The aim of this study was to analyze arrhythmia type and frequency, treatment and recurrence rates [...] Read more.

Background: Arrhythmias are a well known complication in patients with single ventricle physiology (SVP). However, there is still a lack of data regarding arrhythmias in older patients. The aim of this study was to analyze arrhythmia type and frequency, treatment and recurrence rates in patients with SVP over the age of 40 years. Methods: Data was obtained retrospectively from clinical records. All patients > 40 years with SVP with arrhythmias between 2005 and 2018 were included in the study. Treatment was classified as medical, interventional (electrophysiological studies (EPS) in combination with catheter ablation) or direct current cardioversion (DCCV). Results: Altogether, 29 patients (11 female; mean 47.5 ± 4.6 years) with 85 arrhythmia episodes were identified. The median follow-up time was 6.3 years. Cavo-tricuspid (CTI) and non-CTI related intra-atrial reentrant tachycardia (IART) and atrial fibrillation (AF) were most common (48.2% and 37.6%, respectively). In total, 18 EPS/ablations were performed in 9 patients and 52 DCCVs in 20 patients. Acute success was 98% for DCCV and 72.2% for EPS/ablation. Recurrence rate was high (70% for DCCV and 55% for EPS). AT recurrences occurred after a median of 8 and 2.5 months, respectively. On multivariate analyses, age was the only risk factor for arrhythmia recurrence (HR 0.58, 95% C.I. 0.43–0.78, p < 0.0001). Pacemaker implantation was necessary in seven patients (AV block n = 4, sinus node dysfunction n = 3) and one patient received an ICD for secondary prophylaxis. Sudden death occurred in three patients. Conclusions: The most common arrhythmias in patients with SVP > 40 years are IART and AF. Arrhythmia recurrence following EPS or DCCV is frequent. Older age is an independent risk factor for arrhythmia recurrence. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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12 pages, 505 KiB

Open AccessArticle

Association of Health Status Metrics with Clinical Outcomes in Patients with Adult Congenital Heart Disease and Atrial Arrhythmias

by Amalia Baroutidou, Anastasios Kartas, Andreas S. Papazoglou, Diamantis Kosmidis, Dimitrios V. Moysidis, Nikolaos Otountzidis, Ioannis Doundoulakis, Stefanos Despotopoulos, Elena Vrana, Athanasios Koutsakis, Georgios P. Rampidis, Despoina Ntiloudi, Sotiria Liori, Dimosthenis Avramidis, Efstratios Karagiannidis, Theodoros Thomas Nikolopoulos, Sotiria Apostolopoulou, Alexandra Frogoudaki, Afrodite Tzifa, Haralambos Karvounis and George Giannakoulas Show full author list Hide full author list

J. Clin. Med. 2022, 11(20), 6181; https://doi.org/10.3390/jcm11206181 - 20 Oct 2022

Cited by 2 | Viewed by 1692

Abstract

The prognostic value of health status metrics in patients with adult congenital heart disease (ACHD) and atrial arrhythmias is unclear. In this retrospective cohort study of an ongoing national, multicenter registry (PROTECT-AR, NCT03854149), ACHD patients with atrial arrhythmias on apixaban are included. At [...] Read more.

The prognostic value of health status metrics in patients with adult congenital heart disease (ACHD) and atrial arrhythmias is unclear. In this retrospective cohort study of an ongoing national, multicenter registry (PROTECT-AR, NCT03854149), ACHD patients with atrial arrhythmias on apixaban are included. At baseline, health metrics were assessed using the physical component summary (PCS), the mental component summary (MCS) of the Short-Form-36 (SF-36) Health Survey, and the modified European Heart Rhythm Association (mEHRA) score. Patients were divided into groups according to their SF-36 PCS and MCS scores, using the normalized population mean of 50 on the PCS and MCS as a threshold. The primary outcome was the composite of mortality from any cause, major thromboembolic events, major/clinically relevant non-major bleedings, or hospitalizations. Multivariable Cox-regression analyses using clinically relevant parameters (age greater than 60 years, anatomic complexity, ejection fraction of the systemic ventricle, and CHA₂DS₂-VASc and HAS-BLED scores) were performed to examine the association of health metrics with the composite outcome. Over a median follow-up period of 20 months, the composite outcome occurred in 50 of 158 (32%) patients. The risk of the outcome was significantly higher in patients with SF-36 PCS ≤ 50 compared with those with PCS > 50 (adjusted hazard ratio (aHR), 1.98; 95% confidence interval [CI], 1.02–3.84; p = 0.04) after adjusting for possible confounders. The SF-36 MCS ≤ 50 was not associated with the outcome. The mEHRA score was incrementally associated with a higher risk of the composite outcome (aHR = 1.44 per 1 unit increase in score; 95% CI, 1.03–2.00; p = 0.03) in multivariable analysis. In ACHD patients with atrial arrhythmias, the SF-36 PCS ≤ 50 and mEHRA scores predicted an increased risk of adverse events. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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10 pages, 622 KiB

Open AccessArticle

COVID-19 Infections in Adults with Congenital Heart Disease—A Prospective Single-Center Study in an Outpatient Setting

by Nora Langes, Christian Meierhofer, Nicole Nagdyman, Susanne J. Maurer, Felix Bourier, Martin Halle, Stefan Holdenrieder, Peter Ewert and Oktay Tutarel

J. Clin. Med. 2022, 11(20), 6105; https://doi.org/10.3390/jcm11206105 - 17 Oct 2022

Cited by 1 | Viewed by 1244

Abstract

Background: COVID-19 might pose a risk for adults with congenital heart disease (ACHD). However, data regarding the rate of infection as well as myocardial involvement in ACHD patients are currently lacking. Methods: During the study period from January to June 2021, all consecutive [...] Read more.

Background: COVID-19 might pose a risk for adults with congenital heart disease (ACHD). However, data regarding the rate of infection as well as myocardial involvement in ACHD patients are currently lacking. Methods: During the study period from January to June 2021, all consecutive outpatients from our ACHD clinic were eligible to participate. Clinical data were collected. An antibody test for COVID-19 was performed in all patients. Cardiovascular magnetic resonance imaging (CMR) was offered to those with a positive antibody test. Results: Overall, 420 patients (44.8% female, mean age 36.4 ± 11.6 years) participated. Congenital heart defect (CHD) complexity was simple in 96 (22.9%), moderate in 186 (44.3%), complex in 117 (27.9%), and miscellaneous in 21 (5.0%) patients. Altogether, 28 (6.7%) patients had a positive antibody test. Out of these, 14 had an asymptomatic course. The others had mainly mild symptoms and were managed as outpatients. Furthermore, 11 patients (39.3%) had even not been aware of their infection. Fourteen patients underwent a CMR without signs of myocardial involvement in any of them. Conclusions: We observed a number of undetected cases of COVID-19 infections in our ACHD population. Reassuringly, in all cases, the infection had a mild clinical course. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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6 pages, 842 KiB

Open AccessArticle

Self-Reported Mental and Physical Measures in Adult Fontan Patients

by Nili Schamroth Pravda, Oren Zusman, Ilan Richter, Leonard Blieden, Shahar Vig, Ilan Marchushamer, Alexander Dadashev, Yaron Razon, Ran Kornowski and Rafael Hirsch

J. Clin. Med. 2022, 11(14), 3969; https://doi.org/10.3390/jcm11143969 - 8 Jul 2022

Cited by 2 | Viewed by 1510

Abstract

Introduction: The Fontan procedure is a palliative operation for patients with single functional ventricles, arising from a heterogeneous group of heart defects. There is a considerable gap in evidence regarding the self-reported physical and mental health of these patients surviving to adulthood. Methods [...] Read more.

Introduction: The Fontan procedure is a palliative operation for patients with single functional ventricles, arising from a heterogeneous group of heart defects. There is a considerable gap in evidence regarding the self-reported physical and mental health of these patients surviving to adulthood. Methods and Results: We administered the PROMIS^® Global Short Form (v 1.2) to Fontan patients during their scheduled clinic visits during 2017–2018. The raw PROMIS scores were subsequently converted to standardized T-scores, where the mean performance was 50 for the general population. We used Cronbach’s alpha to assess reliability, with >0.8 considered good. A total of 42 patients were included. The median age was 30 (IQR: 24–34) years and 59% (95% CI: 43–74%) were female. The median time from birth to operation was 4.5 (IQR: 3–8) years, with 55% having an extracardiac Fontan. The questionnaire had good internal reliability with an alpha of 0.87. Seventy-one percent of respondents rated their overall health as “excellent” or “good”. The mean T-score for physical health was 46.6, lower than the age-group mean (51.6, p < 0.001). The mean T-score for mental health was 53.3, higher than the age-group mean (48.5, p < 0.001). T-scores showed strong correlation with each other (r = 0.7) and weak correlation with age and time from procedure. There was no association of T-score with diagnosis or operation type. Conclusions: Adult Fontan patients report better mental health despite worse reporting physical health compared with the age group means. Patient-reported measures can provide clinically meaningful insights about the care of patients with complex congenital heart disease. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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11 pages, 1987 KiB

Open AccessArticle

Ambulatory Care in Adult Congenital Heart Disease—Time for Change?

by Louise Coats and Bill Chaudhry

J. Clin. Med. 2022, 11(7), 2058; https://doi.org/10.3390/jcm11072058 - 6 Apr 2022

Cited by 5 | Viewed by 2343

Abstract

Background: The adult congenital heart disease (ACHD) population is growing in size and complexity. This study evaluates whether present ambulatory care adequately detects problems and considers costs. Methods: A UK single-centre study of clinic attendances amongst 100 ACHD patients (40.4 years, median ACHD [...] Read more.

Background: The adult congenital heart disease (ACHD) population is growing in size and complexity. This study evaluates whether present ambulatory care adequately detects problems and considers costs. Methods: A UK single-centre study of clinic attendances amongst 100 ACHD patients (40.4 years, median ACHD AP class 2B) between 2014 and 2019 and the COVID-19 restrictions period (March 2020–July 2021). Results: Between 2014 and 2019, there were 575 appointments. Nonattendance was 10%; 15 patients recurrently nonattended. Eighty percent of appointments resulted in no decision other than continued review. Electrocardiograms and echocardiograms were frequent, but new findings were rare (5.1%, 4.0%). Decision-making was more common with the higher ACHD AP class and symptoms. Emergency admissions (n = 40) exceeded elective (n = 25), with over half following unremarkable clinic appointments. Distance travelled to the ACHD clinic was 14.9 km (1.6–265), resulting in 433–564 workdays lost. During COVID 19, there were 127 appointments (56% in-person, 41% telephone and 5% video). Decisions were made at 37% in-person and 19% virtual consultations. Nonattendance was 3.9%; there were eight emergency admissions. Conclusion: The main purpose of the ACHD clinic is surveillance. Presently, the clinic does not sufficiently predict or prevent emergency hospital admissions and is costly to patient and provider. COVID-19 has enforced different methods for delivering care that require further evaluation. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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23 pages, 399 KiB

Open AccessArticle

Effectiveness and Safety of Transcatheter Atrial Septal Defect Closure in Adults with Systemic Essential Hypertension

by Iwona Świątkiewicz, Łukasz Bednarczyk, Michał Kasprzak, Ewa Laskowska and Marek Woźnicki

J. Clin. Med. 2022, 11(4), 973; https://doi.org/10.3390/jcm11040973 - 13 Feb 2022

Cited by 1 | Viewed by 2501

Abstract

Concomitant systemic essential hypertension (HTN) in adults with a secundum atrial septal defect (ASD) can unfavorably affect the hemodynamics and transcatheter ASD closure (ASDC) effects. This study aims to assess the effectiveness and safety of ASDC in adults with HTN in real-world clinical [...] Read more.

Concomitant systemic essential hypertension (HTN) in adults with a secundum atrial septal defect (ASD) can unfavorably affect the hemodynamics and transcatheter ASD closure (ASDC) effects. This study aims to assess the effectiveness and safety of ASDC in adults with HTN in real-world clinical practice. Right ventricular (RV) reverse remodeling (RVR) and the lack of a left-to-right interatrial residual shunt (NoRS) in echocardiography 24 h and 6 months (6 M) post-ASDC, and ASDC-related complications within 6 M were evaluated in 184 adults: 79 with HTN (HTN+) and 105 without HTN (HTN−). Compared to HTN−, HTN+ patients were older and had a greater RV size and the prevalence of atrial arrhythmias, chronic heart failure, nonobstructive coronary artery disease, diabetes, hyperlipidemia, and left ventricular diastolic dysfunction. ASDC was successful and resulted in RVR, NoRS, and a lack of ASDC-related complications in the majority of HTN+ patients both at 24 h and 6 M. HTN+ and HTN− did not differ in ASD size, a successful implantation rate (98.7% vs. 99%), RVR 24 h (46.8% vs. 46.7%) and 6 M (59.4% vs. 67.9%) post-ASDC, NoRS 24 h (79% vs. 81.5%) and 6 M (76.6% vs. 86.9%) post-ASDC, and the composite of RVR and NoRS at 6 M (43.8% vs. 57.1%). Most ASDC-related complications in HTN+ occurred within 24 h and were minor; however, major complications such as device embolization within 24 h and mitral regurgitation within 6 M were observed. No differences between HTN+ and HTN− were observed in the total (12.7% vs. 9.5%) and major (5.1% vs. 4.8%) complications. Transcatheter ASDC is effective and safe in adults with secundum ASD and concomitant HTN in real-world clinical practice; however, proper preprocedural management and regular long-term follow-up post-ASDC are required. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

11 pages, 487 KiB

Open AccessArticle

Proximal Aortic Dilatation and Pulmonary Valve Replacement in Patients with Repaired Tetralogy of Fallot: Is There a Relationship? A Cardiac Magnetic Resonance Imaging Study

by Ahmed Farghal A. Mohammed, Michael Frick, Gunter Kerst, Nima Hatam, Mohamed-Adel F. Elgamal, Karam M. Essa, Hedwig H. Hövels-Gürich, Jaime F. Vazquez-Jimenez and Rashad Zayat

J. Clin. Med. 2021, 10(22), 5296; https://doi.org/10.3390/jcm10225296 - 15 Nov 2021

Cited by 1 | Viewed by 1962

Abstract

Aortopathy is a known complication whose incidence is growing within the population of tetralogy of Fallot (TOF) patients. Its pathology and relationship with other comorbidities remain unclear. This study was designed to determine the prevalence and predictors of proximal aortic dilatation after TOF [...] Read more.

Aortopathy is a known complication whose incidence is growing within the population of tetralogy of Fallot (TOF) patients. Its pathology and relationship with other comorbidities remain unclear. This study was designed to determine the prevalence and predictors of proximal aortic dilatation after TOF repair. We retrospectively investigated all patients who underwent follow-up cardiac magnetic resonance imaging (CMR; at least 4 years after TOF repair) between March 2004 and December 2019. The dimensions at the ascending aorta (AAo) and sinus of Valsalva (SoV) levels were measured. Aortic dilatation was defined as an internal aortic diameter that was >2 standard deviation of the previously published normal values. We included 77 patients (mean age 28.9 ± 10.5 years, 41.5% female, mean follow-up of 24.5 ± 8.1 years). AAo and SoV were dilated in 19 (24.6%) and 43 (55.8%) patients, respectively. Patients with dilated AAo and SoV were older during the corrective surgery (p < 0.001 and p = 0.004, respectively) and during CMR (p = 0.002 and 0.024, respectively) than patients without AAo and SoV dilatation. Patients of the dilated AAo group were more likely to have prior palliative shunt (p = 0.008), longer shunt duration (p = 0.005), and a higher degree of aortic valve regurgitation (AR) fraction (p < 0.001) and to undergo pulmonary (PVR) and/or aortic valve replacement (p < 0.001 and p = 0.013, respectively). PVR (p = 0.048, odds ratio = 6.413, and 95% CI = 1.013–40.619) and higher AR fraction (p = 0.031, odds ratio = 1.194, and 95% CI = 1.017–1.403) were independent predictors for AAo dilatation. Aortopathy is a common progressive complication that may require reintervention and lifelong follow-up. Our study shows that proximal aortic dilatation may be attributed to factors that increase the volume overload across the proximal aorta, including late corrective surgery and palliative shunt. We also found that PVR and higher AR fraction are independent predictors of AAo dilatation. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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11 pages, 841 KiB

Open AccessArticle

What Is the Importance of Electrocardiography in the Routine Screening of Patients with Repaired Tetralogy of Fallot?

by Paulina Lubocka and Robert Sabiniewicz

J. Clin. Med. 2021, 10(19), 4298; https://doi.org/10.3390/jcm10194298 - 22 Sep 2021

Cited by 4 | Viewed by 1829

Abstract

Background: In patients following complete repair of the tetralogy of Fallot, the duration of the QRS complex is associated with the size and mechanical function of the right ventricle, which are contemporarily assessed by cardiac magnetic resonance (CMR). Methods: 38 patients aged 18.0–54.9 [...] Read more.

Background: In patients following complete repair of the tetralogy of Fallot, the duration of the QRS complex is associated with the size and mechanical function of the right ventricle, which are contemporarily assessed by cardiac magnetic resonance (CMR). Methods: 38 patients aged 18.0–54.9 years (median age 24.9 years) who had undergone complete repair of the tetralogy of Fallot were examined using CMR and concomitant 24 h ambulatory electrocardiography monitoring. We used statistical analysis to investigate the correlations between electrocardiographic parameters (heart rate, HR; PQ interval, PQ; QRS duration, QRS; and corrected QT interval, QTc) and CMR results (right ventricular ejection fraction, RVEF; right ventricular end-diastolic volume index, RVEDVI; and right ventricular end-systolic volume index, RVESVI) for patients after early and late repair. Results: The ECG-based parameters were not correlated with time since repair. There were significant correlations between QRS duration and RVEF (r = −0.61), RVEDVI (r = 0.56), and RVESVI (r = 0.54) for early operated patients but not for late-operated patients. No other substantial correlations were reported. Conclusion: Despite its role in screening for arrhythmias, electrocardiography has a limited role as a predictor of morphology and function of the right ventricle in patients after repair of the tetralogy of Fallot. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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9 pages, 814 KiB

Open AccessArticle

Complete Atrioventricular Septal Defects after the Age of 40 Years

by Susanne J. Maurer, Lorena Moosholzer, Claudia Pujol, Nicole Nagdyman, Peter Ewert and Oktay Tutarel

J. Clin. Med. 2021, 10(16), 3665; https://doi.org/10.3390/jcm10163665 - 19 Aug 2021

Cited by 2 | Viewed by 1351

Abstract

Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years. Methods: Patients [...] Read more.

Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years. Methods: Patients with cAVSD who were ≥40 years of age at any point between 2005 until 2018 were included retrospectively. Data were retrieved from hospital records. The primary endpoint was a combination of death from any cause and unplanned hospitalizations due to cardiac reasons. Results: 43 patients (60.5% female, mean age 43.7 ± 6.0 years, genetic syndrome 58.1%) were included. At begin of follow-up, the majority of patients (n = 41, 95.3%) was in New York Heart Association (NYHA) class I or II. Out of the whole cohort 26 (60.5%) had undergone cardiac surgery. At baseline, at least one extracardiac comorbidity was present in 40 patients (93.0%). Median follow-up was 1.7 years (IQR 0.3–4.6). On univariate Cox analysis, NYHA class at begin of follow-up (hazard ratio: 1.96, CI 95%: 1.04–3.72, p < 0.05) was the only predictor for the primary endpoint. Conclusions: Significant morbidity and mortality is present in cAVSD patients over the age of 40 years. NYHA class is predictive for a worse outcome. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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10 pages, 361 KiB

Open AccessArticle

Acquired Comorbidities in Adults with Congenital Heart Disease: An Analysis of the German National Register for Congenital Heart Defects

by Susanne J. Maurer, Ulrike M. M. Bauer, Helmut Baumgartner, Anselm Uebing, Claudia Walther and Oktay Tutarel

J. Clin. Med. 2021, 10(2), 314; https://doi.org/10.3390/jcm10020314 - 16 Jan 2021

Cited by 14 | Viewed by 2981

Abstract

Background: As adults with congenital heart disease (ACHD) are getting older, acquired comorbidities play an important role in morbidity and mortality. Data regarding their prevalence in ACHD that are representative on a population level are not available. Methods: The German National Register for [...] Read more.

Background: As adults with congenital heart disease (ACHD) are getting older, acquired comorbidities play an important role in morbidity and mortality. Data regarding their prevalence in ACHD that are representative on a population level are not available. Methods: The German National Register for Congenital Heart Defects was screened for ACHD. Underlying congenital heart disease (CHD), patient demographics, previous interventional/surgical interventions, and comorbidities were retrieved. Patients <40 years of age were compared to those ≥40 years. Results: A total of 4673 patients (mean age 33.6 ± 10.7 years, female 47.7%) was included. At least one comorbidity was present in 2882 patients (61.7%) altogether, and in 56.8% of patients below vs. 77.7% of patients over 40 years of age (p < 0.001). Number of comorbidities was higher in patients ≥40 years (2.1 ± 2.1) than in patients <40 years (1.2 ± 1.5, p < 0.001). On multivariable regression analysis, age and CHD complexity were significantly associated with the presence and number of comorbidities. Conclusions: At least one acquired comorbidity is present in approximately two-thirds of ACHD. Age and complexity of the CHD are significantly associated with the presence of comorbidities. These findings highlight the importance of addressing comorbidities in ACHD care to achieve optimal long-term outcomes. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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8 pages, 394 KiB

Open AccessArticle

Patients with Single-Ventricle Physiology over the Age of 40 Years

by Claudia Pujol, Sandra Schiele, Susanne J. Maurer, Julia Hock, Celina Fritz, Alfred Hager, Peter Ewert and Oktay Tutarel

J. Clin. Med. 2020, 9(12), 4085; https://doi.org/10.3390/jcm9124085 - 18 Dec 2020

Cited by 12 | Viewed by 2761

Abstract

Background: Single-ventricle physiology (SVP) is associated with significant morbidity and mortality at a young age. However, survival prospects have improved and risk factors for a negative outcome are well described in younger cohorts. Data regarding older adults is scarce. Methods: In this study, [...] Read more.

Background: Single-ventricle physiology (SVP) is associated with significant morbidity and mortality at a young age. However, survival prospects have improved and risk factors for a negative outcome are well described in younger cohorts. Data regarding older adults is scarce. Methods: In this study, SVP patients under active follow-up at our center who were ≥40 years of age at any point between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history were retrieved from hospital records. The primary end-point was all-cause mortality. Results: Altogether, 49 patients (19 female (38.8%), mean age 49.2 ± 6.4 years) were included. Median follow-up time was 4.9 years (interquartile range (IQR): 1.8–8.5). Of these patients, 40 (81.6%) had undergone at least one cardiac surgery. The most common extracardiac comorbidities were thyroid dysfunction (n = 27, 55.1%) and renal disease (n = 15, 30.6%). During follow-up, 10 patients (20.4%) died. On univariate analysis, renal disease and liver cirrhosis were predictors of all-cause mortality. On multivariate analysis, only renal disease (hazard ratio (HR): 12.5, 95% confidence interval (CI): 1.5–106.3, p = 0.021) remained as an independent predictor. Conclusions: SVP patients ≥40 years of age are burdened with significant morbidity and mortality. Renal disease is an independent predictor of all-cause mortality. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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7 pages, 420 KiB

Open AccessFeature PaperArticle

Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years

by Susanne J. Maurer, Katharina Stöckemann, Claudia Pujol, Jürgen Hörer, Peter Ewert and Oktay Tutarel

J. Clin. Med. 2020, 9(12), 4071; https://doi.org/10.3390/jcm9124071 - 17 Dec 2020

Cited by 7 | Viewed by 2236

Abstract

Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking. Methods: PAH-ACHD patients ≥ 40 years of age under active follow-up between January [...] Read more.

Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking. Methods: PAH-ACHD patients ≥ 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality. Results: In total, 65 patients (67.7% female, mean age 45.19 ± 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2–7.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2–14.4, p = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2–118.7, p = 0.006) remained as independent predictors of all-cause mortality. Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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8 pages, 382 KiB

Open AccessArticle

Comparison of the Change in Diastolic Dysfunction after Transcatheter Atrial Septal Defect Closure between Asymptomatic Younger and Older Age Groups

by Lucy Youngmin Eun, Han Ki Park and Jae Young Choi

J. Clin. Med. 2020, 9(11), 3637; https://doi.org/10.3390/jcm9113637 - 12 Nov 2020

Cited by 2 | Viewed by 1991

Abstract

Transcatheter device closure of a secundum atrial septal defect (ASD) is followed by a noticeable change in the left atrium (LA) and left ventricle (LV) over a long-term follow-up. This study aimed to assess the remodeling of the atrial and ventricular myocardium with [...] Read more.

Transcatheter device closure of a secundum atrial septal defect (ASD) is followed by a noticeable change in the left atrium (LA) and left ventricle (LV) over a long-term follow-up. This study aimed to assess the remodeling of the atrial and ventricular myocardium with respect to diastolic function in adult ASD patients. Around 38 asymptomatic patients (age: 48.6 ± 17.1 years, range: 23–69 years) diagnosed with ASD during routine health check-up and who underwent ASD device closure with the Amplatzer septal occluder were included in this retrospective study, and their medical records (containing echocardiographic data) were reviewed. The defect size was 21.77 + 6.79 mm, while the balloon measurement of the defect was 24.29 ± 6.64 mm. The patients were divided into two groups, namely, Group I (with 18 patients aged <50 years [mean: 33.06 ± 9.43 years] and Group II (with 20 patients aged ≥50 years [mean: 62.55 ± 7.54 years]). Comparison of echocardiographic data collected before closure and at a follow-up 2 years later revealed significant differences between pre-closure and post-closure states in the left ventricular end-diastolic dimension (40.76 ± 3.28 vs. 43.39 ± 3.52), left ventricular mass (99.64 ± 28.81 vs. 116.57 ± 32.03), and right ventricular pressure (36.88 ± 12.20 vs. 31.81 ± 11.11). Tissue Doppler measurements were significantly decreased 2 years after closure, while the post-closure E/E’ was higher than the pre-closure E/E’ (11.58 ± 4.80 vs. 8.79 + 3.19, p < 0.005). In both groups, mitral A and tissue Doppler E’, A’, and S’ values decreased post-closure, while the E/E’ was higher in Group II than in Group I at both pre-closure and long-term follow-up measurements (pre-closure: 9.60 ± 5.15 vs. 7.41 ± 1.42, p < 0.003; post-closure: 13.03 + 4.05 vs. 10.49 ± 3.95, p < 0.02). The LA size and LV function exhibited recognizable remodeling after transcatheter ASD closure. Because the LV preload elevation (i.e., E/E’) after ASD closure can be pronounced in older patients, caution should be provided more in older patients than in younger patients. Hence, it may be beneficial to close the ASD at an earlier age in adults even after a late diagnosis; a relatively younger age may be more suited for adaptation to the remodeling process to protect the myocardial function. Careful consideration should be given to the previous underloaded left heart after long-term closure. Full article

(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population)

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Adult Congenital Heart Disease: An Increasing and Ageing Population

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