• Korean Circulation Journal
• /
• 제52권3호
• /
• pp.233-242
• /
• 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
• /
• 제50권11호
• /
• pp.1055-1060
• /
• 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
• /
• 제22권1호
• /
• pp.95-105
• /
• 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.

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

• 대한두개안면성형외과학회지
• /
• 제19권3호
• /
• pp.235-239
• /
• 2018

Intraoperative expansion has been used to cover small to large defects without disadvantages of the conventional tissue expanders. Various materials, for example, expanders and Foley catheters are being used. We introduce a new, convenient and economical device immediately available in the operating room, according to the defect size for intraoperative expansion, with latex gloves or balloons. The retrospective study was done with 20 patients who presented with skin and soft tissue defects. During the operation, expansion was done with latex gloves or balloons inflated with saline through an intravenous line and a three-way stopcock. After the inflation, the glove was removed and skin was covered with expanded tissue. A careful decision was made regarding the inflation volume and placement of the expander according to the defect size. There were no postoperative complications. The skin contracture and tension was minimal with a texture similar to the adjacent tissue. The new intraoperative expansion devices with latex gloves and balloons were cheap and made easily right in the operation room. The reconstruction of small to large sized skin defects can be done successfully, functionally and aesthetically without using expensive commercial materials.

• Journal of Chest Surgery
• /
• 제22권6호
• /
• pp.990-995
• /
• 1989

Since 1984, 24 patients underwent repair of atrioventricular septal defect. Nineteen had a partial defect and 5 had a complete atrioventricular septal defect. There were 9 men and 15 women, ranging in age from 1 to 50 years [mean age, 13.3 years]. Four patients had a Downs syndrome. Additional congenital heart defects were present in 11 patients. One patient had palliative operation prior to total correction. In partial defects, the primum atrial septal defect was closed with Xenomedica patch and the mitral valve was repaired with simple closure of the septal commissure. Central incompetence from annular dilatation was repaired by a local annuloplasty. In complete defect, the septal defects were closed with two patches except one. Operative mortality was 5% in partial defects and 60% in complete defects and low cardiac output was the commonest etiology. In a mean follow-up period of 27.9 months [range, 4 to 63 months] there were no late death and no instances of late-onset complete heart block. One patient required reoperation [MVR] for residual mitral regurgitation. The majority of patients were asymptomatic and mean postop. NYHA functional class was 1.2.

• Journal of Chest Surgery
• /
• 제16권1호
• /
• pp.75-82
• /
• 1983

This report is concerned to our experience of 10 cases of open heart surgery under the extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery, Capital Armed Forces General Hospital during the period between May, 1982 and February, 1983. 1. Six cases were male and two cases were female. Age was varied from 21 years to 50 years and mean age was 34 years. 2. The cases included 2 Ventricular Septal Defects, 1 Atrial Septal Defect, I Tetralogy of Fallot and 6 acquired valvular heart diseases. 3. The surgical managements were 3 primary repairs for Ventricular Septal Defects and Atrial Septal Defect, I total correction for Tetralogy of Fallot and 6 mitral valve replacements with bovine xenograft by Ionescu-Shiley combining 3 Tricuspid annuloplasties [ De Vega method ] and 1 deauricularization of left atrial appendage for acquired valvular heart diseases. 4. The average cardiopulmonary bypass time was 37 minutes for acyanotic congenital heart diseases and 92 minutes for cyanotic heart disease and acquired valvular heart diseases. And the average aortic cross clamping time was 19 minutes for the former and 70 minutes for the latter. 5. Postoperatively, there were 1 hemolytic anemia, 1 congestive heart failure, 1 hemolytic jaundice and 1 thermal burn as complications, but there was no operative mortality. 6. All patients received valve replacement were recommended anticoagulation with Persantin and Aspirin.

• Journal of Chest Surgery
• /
• 제11권1호
• /
• pp.46-57
• /
• 1978

Ventricular septal defect was the most common congenital Heart disease admitted to the Severance Hospital from December, 1963 to June 30. 1977. A total of 1,811 cardiac patients received cardiac catheterization in the CardiacCenter of Yonsei University Medical College. Out of the 1,811 cardiac patients, 791 cases had congenital heart disease and of these 172 cases had ventricular septal defect. This amounted to 21.7% of all those with congenital heart disease. Seventy cases of ventricular septal defect operated on in the chest surgery department are presented. Of these 70 cases of ventricular septal defect, 54 cases were male and 16 cases were female. Their ages ranged from 4/12 months to 32 years. The ratio of systolic pressure of the main pulmonary to systemic artery [Pp/Ps] for 29 of the 59 isolated ventricular septal defects was below 45 percent. Pp/Ps ratio for 19 of the 59 isolated ventricular septal defects was 75 percent. The patients were mostly below fifteen years of age. Out of 64 cases, there were 36 cases of type II defects, 20 cases of type I, 4 cases of type III , 2 cases of type IV, one case of both type II and one case of left ventricular right atrial communication. The anomalies associated with ventricular septal defect were 13 in all; 4 cases of aortic insufficiency, 3 cases of ruptured aneurysm of the sortie sinus of valsalva, 2 cases of ruptured aneurysm of the sortie sinus of valsalva with aortic insufficiency, 2 cases of patent ductus arteriosus, one case of ruptured aneurysm of the aortic sinus of valsalva with atrial septal defect [secundum type] and one case of atrial septal defect [secundum type]. Overall mortality was 8.6 percent. The mortality in pulmonary artery banding was 37.5 percent. The causes of mortality were in one case congestive heart failure, in one case asphyxia and in one case respiratory insufficiency. Tile mortality in ventricular septal defect associated with pulmonary hypertension was 7.1 percent. The cause of mortality was in one case low cardiac output syndrome. There was no mortality in the ventricular septal defects without pulmonary hypertension and mortality in the ventricular septal defect. In ventricular septal defect associated with combined anomalies, the causes mortality were in one case respiratory insufficiency and in one case congestive heart failure.

• 대한전기학회:학술대회논문집
• /
• 대한전기학회 2011년도 제42회 하계학술대회
• /
• pp.2051-2052
• /
• 2011

The structural defects of a heart often reflects the sounds that the heart produces. This paper describes heart sound analysis method using Wavelet transform and average Shannon energy. This can extract the features of heart sounds in various disease identify the heart sounds. Experimental results show that the presented method has potential application in detecting various heart diseases.

• Clinical and Experimental Pediatrics
• /
• 제53권6호
• /
• pp.680-687
• /
• 2010

Patients with congenital heart diseases (CHD) are confronted with early- and late-onset complications, such as conduction disorders, arrhythmias, myocardial dysfunction, altered coronary flow, and ischemia, throughout their lifetime despite successful hemodynamic and/or anatomical correction. Rhythm disturbance is a well-known and increasingly frequent cause of morbidity and mortality in patients with CHD. Predisposing factors to rhythm disturbances include underlying cardiac defects, hemodynamic changes as part of the natural history, surgical repair and related scarring, and residual hemodynamic abnormalities. Acquired factors such as aging, hypertension, diabetes, obesity, and others may also contribute to arrhythmogenesis in CHD. The first step in evaluating arrhythmias in CHD is to understand the complex anatomy and to find predisposing factors and hemodynamic abnormalities. A practical stepwise approach can lead to diagnosis and prompt appropriate interventions. Electrophysiological assessment and management should be done with integrated care of the underlying heart defects and hemodynamic abnormalities. Catheter ablation and arrhythmia surgery have been increasingly applied, showing increasing success rates with technological advancement despite complicated arrhythmia circuits in complex anatomy and the difficulty of access. Correction of residual hemodynamic abnormalities may be critical in the treatment of arrhythmia in patients with CHD.