Repaired and open atrial septal defects type II in adulthood: An epidemiological study of a large European cohort

Engelfriet, Peter; Meijboom, Folkert J.; Boersma, Eric; Tijssen, Jan G.P.; Mulder, Barbara J.M.

doi:10.1016/j.ijcard.2007.04.044

Cited by 71 publications

(53 citation statements)

References 13 publications

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“…Pulmonary hypertension complicating ASD is encountered in 6-35% of untreated patients in later adulthood. 6 The presence of PH causes progressive dyspneu, presyncope, syncope, edema and functional limitations. 7 Furthermore, right ventricular volume overload and right ventricular dysfunction generate more progressive sign and symptom.…”

Section: Discussionmentioning

confidence: 99%

“…7 Furthermore, right ventricular volume overload and right ventricular dysfunction generate more progressive sign and symptom. 6 Palpitation is possibly the indication of impending heart failure or arrhytmia. Supraventricular arrhythmia, due to stretching of the conduction system, may be the first presenting sign of an ASD.…”

Section: Discussionmentioning

confidence: 99%

“…8 In patients with uncorrected ASD, the clinical parameters tend to worsen over time. 6 Nevertheless, a substantial number of patients with open ASD have normal parameters, usually the patients with small defects in whom the hemodynamic parameters remained stable during several years of follow up. 6 In our cohorts, despite developing PH, around 6% of patients come in later life (sixth decade).…”

Section: Discussionmentioning

confidence: 99%

“…6 Nevertheless, a substantial number of patients with open ASD have normal parameters, usually the patients with small defects in whom the hemodynamic parameters remained stable during several years of follow up. 6 In our cohorts, despite developing PH, around 6% of patients come in later life (sixth decade). The long period of asymptomatic or mild symptom may be associated with small ASD.…”

Section: Discussionmentioning

confidence: 99%

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Clinical characteristics of adult uncorrected secundum atrial septal defect: a pilot study

et al. 2016

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Atrial septal defect (ASD) is the most frequent congenital heart disease in adulthood. Pulmonary hypertension (PH) complicating ASD compels patients seeking medical assistance because of its disabling symptom. Most adult ASD develop PH which render significant morbidity and mortality. The aim of the study is to characterize the clinical profiles of adult patients with ASD. The study design was cross sectional. The subjects were enrolled consecutively from outpatient clinics and inpatient wards. The demography, medical and imaging data were collected and recorded in case report form. Descriptive statistics was applied to characterize the subjects. Seventy-six subjects were enrolled. The majority were women (77.6 %) in the productive and child-bearing ages (63.2%). The most common symptoms were dyspneu on effort, fatigue, and palpitation. Most subjects had functional capacity of WHO class functional II (70.2 %). The mean oxygen saturation was 96.4 %. Based on the echocardiography examination, 77.6% of subjects had suffered from PH. The mean longest diameter of defects were 2.7 cm. The direction of blood flow was mostly left to right (77.6 %). Left and right ventricle function were within normal limit. Right heart catheterization showed mean left atrial pressure 11.5 mmHg, which confirmed the precapillary or arterial PH. Mean pulmonary artery pressure was 42.0 mmHg. The pulmonary artery resistance index mostly less than 4 Wood Unit/ m 2 (63.7 %), indicating the feasibility to close the defect. Whereas 24.6 % of subjects were contraindicated for closing. Pulmonary artery hypertension (PAH) was diagnosed in 77.6 % subjects, meanwhile 13.2 % had borderline PAH. In conclusion, most adult ASD patients had developed PAH, mostly young women in productive ages, mainly visited hospital due to symptom of PH, the direction of flow predominantly left to right side and mostly had reduced functional capacity. ABSTRAKDefek septum atrium (DSA) merupakan penyakit jantung bawaan yang paling sering dijumpai pada usia dewasa karena tanda dan gejalanya yang samar. Hipertensi paru (HP) merupakan komplikasi DSA yang mendorong pasien ke rumah sakit karena munculnya gejala yang berat. Hampir semua pasien DSA dewasa mengalami HP yang menyebabkan angka kesakitan dan kematian bermakna. Tujuan penelitian ini adalah untuk mengkaji karakteristik klinis pasien dengan DSA dan HP. Desain penelitian adalah potong lintang. J Med Sci, Volume 48, No. 2, 2016 April: 89-97 90Subjek diseleksi secara berurutan dari klinik rawat jalan dan rawat inap. Data demografi, medis dan pencitraan dikumpulkan dan dicatat dalam suatu blangko laporan kasus. Penelitian deskriptif dilakukan untuk menilai karakteristik subjek. Sebanyak tujuh puluh enam subjek dilibatkan dalam penelitian. Sebagian besar subjek adalah wanita (77,6%) usia muda, produktif dan subur (63,2%). Gejala paling sering adalah sesak saat aktivitas, mudah lelah, dan berdebar. Sebagian besar pasien masuk dalam kelas fungsional WHO II (70,2%). Rerata saturasi oksigen 96,4%. Berdasar hasil ekok...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Clinical characteristics of adult uncorrected secundum atrial septal defect: a pilot study

et al. 2016

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“…The CHD-PAH group is a well defined population which is investigated and compared to other groups thoroughly. [4][5][6] New in the updated clinical classification of PAH is the subdivision of CHD-PAH in four main groups: 1) Eisenmenger syndrome; 2) PAH associated with systemic-to-pulmonary shunts; 3) PAH with small defects; and 4) PAH after corrective cardiac surgery. The pathophysiologic mechanism for all these four groups involves intracardiac shunting and increased flow, though in different stages.…”

Section: Introduction To the Management Of Pulmonary Arterial Hypertementioning

confidence: 99%

Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan

Schuuring

Vis²,

Duffels³

et al. 2010

TCRM

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Pulmonary arterial hypertension (PAH) is a progressive disease with poor survival outcome. PAH is classified by the 2009 updated clinical classification of pulmonary hypertension and a major subgroup is PAH due to congenital heart disease (CHD) with systemic-to-pulmonary shunt. CHD-PAH is a result of systemic-to-pulmonary shunting and chronic increased flow that ultimately results in adaptations of pulmonary vasculature and endothelial dysfunction. The advanced stage is called Eisenmenger syndrome which forms a small percentage (1%) of all CHD patients. Therapies targeted on PAH symptoms are called primary therapy for PAH, but most CHD-PAH patients progress to advanced therapy which is directed at the PAH itself. In CHD-PAH, advanced therapies are extensively investigated for all three major pathways: endothelin-1 receptor antagonists such as bosentan, prostanoids such as epoprostenol and phosphodiesterase 5 inhibitors such as sildenafil. Endpoints in most trials were catheterization hemodynamics, World Health Organization functional class, six-minute walking distance and patient-focused outcomes, based on quality of life questionnaires and Borg dyspnea index. The BREATHE-5 and EARLY study were two important randomized controlled trials showing efficacy of bosentan at short follow-up. Moreover in patients with Eisenmenger syndrome, one recent survival retrospective study with majority of patients on bosentan showed strong survival benefit over conservative therapy. A diversity of prospective cohort and retrospective studies were performed but all with limited data, due to small numbers and heterogeneity of underlying CHD diagnoses. Further larger studies are needed to determine optimal treatment for adults with CHD-PAH. This review focuses on bosentan in CHD-PAH. In particular, we discuss outcome of various clinical trials and compare efficacy and safety of bosentan to other advanced therapies.

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Anomalies of the Atrial Septum

Geva¹

2016

Echocardiography in Pediatric and Congenital Heart Disease

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Repaired and open atrial septal defects type II in adulthood: An epidemiological study of a large European cohort

Cited by 71 publications

References 13 publications

Clinical characteristics of adult uncorrected secundum atrial septal defect: a pilot study

Clinical characteristics of adult uncorrected secundum atrial septal defect: a pilot study

Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan

Anomalies of the Atrial Septum

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