• Korean Circulation Journal
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• 제52권3호
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• pp.233-242
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• 2022

Background and Objectives: For adult men with congenital heart disease (ACHD), data on erectile dysfunction (ED) is limited. We aimed to assess the frequency of ED, its role in patient-physician communication and to identify parameters predicting ED. Methods: Male ACHD ≥18 years registered at the German National Register for Congenital Heart Defects were invited to participate in an online questionnaire about sexual health. Participants with presumed ED according to International Index of Erectile Function Score were compared to patients without ED. Results: The 371 patients responded to the questionnaire (83% with moderate to highly complex ACHD). The 43% presented with more than mild ED. When ED was present, patients complained about general anxiety to be sexually active more often (p<0.05) and underwent sexual activity less frequently compared to those without ED (p<0.05). Age ≥40 years (odds ratio [OR], 3.04; p=0.002), being single (OR, 6.82; p<0.0001), anxiety to be sexually active (OR, 2.64; p=0.0002) and psychiatric disease (OR, 4.33; p<0.0007) emerged as independent predictors for ED. Overall, patients sought medical advice in 6.7% of cases, whilst 29.6% would appreciate an active approach by the physician to address this sensitive topic. Conclusions: ED is affecting one third to one half of male ACHD according to a questionnaire-based analysis. Older age, being single, fear of sexual activity due to ACHD and psychiatric disorder emerged as independent predictors for ED. These parameters can easily be assessed to identify patients at risk. ED should be addressed proactively by health professionals.

• Clinical and Experimental Pediatrics
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• 제50권11호
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• pp.1055-1060
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• 2007

Right-sided heart failure is a major problem among patients with congenital heart diseases, due to the prevalence of congenital heart defects and the association of pulmonary hypertension. More attention is focused on the structure of the right heart particularly in association with congenital heart defects and chronic lung disease. The right ventricle (RV) may support the pulmonary circulation, and sometimes the systemic circulation (systemic RV) in congenital heart defects. Despite major progress being made, assessing the RV remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and cineangiography). Evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. While there is extensive literature on the pathophysiology and treatment of left heart failure, the data for right-sided heart failure is scarce. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. An understanding of RV physiology and hemodynamics will lead to a better understanding of current and future treatment strategies for right heart failure. This will review right-sided heart failure with the implications of volume and pressure loading of the RV in congenital heart diseases.

• Journal of Chest Surgery
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• 제14권1호
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• pp.87-90
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• 1981

Genetic and environmental factors are the two areas which have received attention in the etiology of congenital cardiac malformation. Genetic factor in many types of congenital heart disease have not been clearly delineated. Congenital heart diseases are a heterogenous category of developmental anomalies, representing in most cases the multifactorial inheritance of threshold characters, the expression of which is the product of a genetic - environmental interaction. Recently we experienced three pairs of congenital heart disease in siblings including ventricular septal defects in twin.

• Clinical and Experimental Pediatrics
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• 제64권6호
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• pp.269-279
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• 2021

Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) is an ancient prokaryotic defense system that precisely cuts foreign genomic DNA under the control of a small number of guide RNAs. The CRISPR-Cas9 system facilitates efficient double-stranded DNA cleavage that has been recently adopted for genome editing to create or correct inherited genetic mutations causing disease. Congenital heart disease (CHD) is generally caused by genetic mutations such as base substitutions, deletions, and insertions, which result in diverse developmental defects and remains a leading cause of birth defects. Pediatric CHD patients exhibit a spectrum of cardiac abnormalities such as septal defects, valvular defects, and abnormal chamber development. CHD onset occurs during the prenatal period and often results in early lethality during childhood. Because CRISPR-Cas9-based genome editing technology has gained considerable attention for its potential to prevent and treat diseases, we will review the CRISPR-Cas9 system as a genome editing tool and focus on its therapeutic application for CHD.

• Child Health Nursing Research
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• 제25권3호
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• pp.262-272
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• 2019

Purpose: We aimed to identify factors influencing physical activity in adolescents with complex congenital heart disease. Methods: We recruited 92 adolescents with complex congenital heart disease from a tertiary medical center in Seoul, measured their levels of physical activity, and identified factors that influenced their physical activity levels using the Global Physical Activity Questionnaire, the New York Heart Association classification, congenital heart disease complexity, the Self-Efficacy Scale, and the Parental Bonding Instrument scale. Stepwise multiple linear regression was used to determine factors influencing physical activity. Results: Total physical activity was higher in males than in females (t=4.46, p<.001). Adolescents who participated in school physical education classes engaged in more physical activity than those who did not (t=6.77, p<.001). Higher self-efficacy (${\beta}=.41$, p<.001), male gender (${\beta}=.37$, p<.001) and participation in school physical education classes (${\beta}=.19$, p=.042) were associated with a higher likelihood of engagement in physical activity. Conclusion: It is necessary to develop nursing interventions that enhance self-efficacy in order to promote physical activity in adolescents with complex congenital heart disease. Physical activity should also be promoted in an individualized manner, taking into account gender, disease severity, and parental attitude.

• Journal of Chest Surgery
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• 제22권1호
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• pp.95-105
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• 1989

The records of 248 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular malformation during the period of 10 years from August, 1978 to July, 1988 were reviewed. During this period, the incidence of congenital cardiovascular malformation in adult was 18.2% of 1376 total heart disease operated on and 25.5% of 986 congenital heart defects. Among them, there were 200 patients in acyanotic group and 48 patients in cyanotic group. Male versus female ratio was 1:1.28. The oldest patient was 59 years old female who had atrial septal defect. The mean age was 24.4 years old. The distribution of the lesions showed a large preponderance of atrial septal defects [37.19o] followed in frequency by ventricular septal defects [918.1%], patent ductus arteriosus [17.3%], tetralogy of Fallot [16.1%], and a variety of other complex malformations[3.2%]. In the pediatric age group, relative frequency was different from that of this adult group, showing ventricular septal defects, tetralogy of Fallot, patent ductus arteriosus and atrial septal defects in order of incidence. The hospital mortality and late mortality were 6.0% and 1.7% respectively. The causes of hospital death were low cardiac output in 10 patients, arrhythmia in 2, air embolism in 1, sepsis in 1 and respiratory failure in l. Clinical improvement upto NYHA functional class I or II postoperatively has been achieved and sustained in all patients following repair except the patients of late death and receiving reoperation. This result confirms that congenital heart defects in the adults can be corrected with a good outcome and an aggressive operative approach seems justified.

• Korean Journal of Radiology
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• 제22권11호
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• pp.1894-1908
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• 2021

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

• Journal of Chest Surgery
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• 제21권1호
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• pp.48-54
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• 1988

168 cases of open heart surgery had been performed in Korea Veterans Hospital from Aug. 1984 to Nov. 1987. There were 150 cases of congenital heart disease and 18 cases of acquired heart disease. In congenital heart cases, 123 cases [82%] were acyanotic and 27 cases [18%] were cyanotic. Common congenital defects were VSD, TOF, ASD, PS in order of frequency. There were 11 cases of operative mortality; 5 cases [4%] in acyanotic group, 6 cases [22\ulcorner6] in cyanotic group. There was no mortality in valvular heart disease group. Overall mortality rate was 6.5%.

• Journal of Chest Surgery
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• 제29권9호
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• pp.940-944
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• 1996

경북대학교 병원에서는 1990년 1월부터 1994년 10월까지 그 연령이 15세 이상인 선천성 심장병 환자 를 수술적 치료로 교정하였다 이 기간동안 총 628례의 선천성 심장병 환자중 22.4%인 143명이 성인 환자였다. 그중 10대가 23례, 20대가 58례, 30대가 34례, 40대가 18례, 50대가 10례였다. 가장 많은 질환은 전체의 51.1 % (73례)를 차지한 심방중격결손이었고, 39.9 % (57례)의 심실중격결손, 2.8 % (4례)의 활로씨 4징후순이었다. 술후 합병증은 10례 (6.9 %)에서 있었고 수술 사망은 없었다 이상의 결과로 성인에 있어서 수술이 가능한 예의 빈도를 알수 있었으며,이러한 경우에는적은수술 사망과 술후 합병증으로 수술이 가능하였다.

• Journal of Chest Surgery
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• 제19권1호
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• pp.116-121
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• 1986

Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.