• Journal of Chest Surgery
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• 제19권2호
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• pp.306-312
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• 1986

The development of extracardiac valved conduits for establishment of RV-PA continuity has permitted repair of previously uncorrectable congenital heart anomalies and has facilitated the repair of other complex lesions. But the distressing problem of neointimal peel formation with eventual conduit obstruction in patients with Dacron valved conduits has led to the need for premature replacement in many patients. Therefore in the absence of pulmonary hypertension, hypoplastic pulmonary arteries, significant right ventricular dysfunction or unrepaired tricuspid regurgitation, preference of a valveless conduit to a valved conduit gives excellent results and may diminish the need for late reoperation. In our Hanyang University Hospital, the Rastelli operation was performed for the repair of pulmonary atresia with a valveless Gore-Tex conduit. The patient was operated on with good result. The CVP after operation was 8-13 mmHg at POD #0 and 4-6 mmHg from POD #3-4. Postoperatively the patient was acyanotic and had improved physical capacity compared with his preoperative status.

• Journal of Chest Surgery
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• 제17권2호
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• pp.280-285
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• 1984

Although it is suspected that the foreign body sensation on the pharyngoesophageal region is caused by motility disturbance of upper esophageal sphincter, its pathophysiology is not yet clear. Esophageal manometry has become an important diagnostic tool in the evaluation of esophageal motor disorders such as dysfunction of upper esophageal sphincter. Intraluminal esophageal pressures were measured by perfusion manometry in fifteen patients with foreign body sensation on the pharyngoesophageal region and in twenty six controls. In upper esophageal sphincter, mean value of resting pressure of the patients by rapid pull-through technique was 45,9\ulcorner 15.6mmHg and 80.9\ulcorner9.7mmHg in the controls. The difference between the two groups was statistically significant. The distance from nostril to sphincter, length of sphincter, and resting pressure by station pull-through technique were not significantly different. The amplitude of esophageal peristalsis in the patients was reduced significantly at the level of the upper, mid and lower esophagus. The wave duration of the patients was reduced significantly at the level the upper and mid esophagus. The speed showed no difference between two groups. Length and resting pressure of lower esophageal sphincter revealed almost same values in two groups.

• Journal of Chest Surgery
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• 제17권4호
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• pp.672-677
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• 1984

Thromboembolism is a major cause of morbidity and death following implantation of cardiac prosthetic devices. Effective systemic anticoagulation is very important. The presence of thrombus can often be detected by pulmonary edema associated with the disappearance of valve clicks. 2-D echocardiography and phonocardiography are also valuable tools. The most common treatment is reoperation and replacement after discovery early. We have experienced one death of fatal thromboembolism after St. Jude valve replacement was done in 48 years old male diagnosed of severe mitral stenosis. He was treated with warfarin, aspirin, ticlopidine for 10 weeks but died suddenly. At autopsy, valve dysfunction was seen due to organic thrombus arising from atrial septum and confirmed with microscopic findings.

• Journal of Chest Surgery
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• 제20권2호
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• pp.317-322
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• 1987

Records of 15 patients who underwent operation for constrictive pericarditis at this department from 1976 through 1984 were reviewed. All had hemodynamically significant pericardial constriction preoperatively, and pericardial disease was confirmed at operation. There were 12 males and 3 females in this series. Range of age varied from 7 years to 51 years. Clinical and histological study revealed granulomatous pericarditis compatible with the diagnosis of tuberculosis in 5 patients, non-specific chronic inflammatory changes in 6 patients and pyogenic pericarditis in 2 patients. Pericardiectomy was performed through a median sternotomy [11 cases] or bilateral anterior thoracotomy [4 cases]. The postoperative complications were observed in 4 patients. Low cardiac output was the most common complication [2 patients]. In 13 cases, excluding 2 operative deaths, preoperatively all were in New York Heart Association Class III or IV. At the time of discharge II [85%] were in Class I or II. 4 Cases were reoperated after original pericardiostomy and all resulted in marked improvement. Early surgical intervention is advisable in all patients in whom cardiac constriction is caused by either a thickened pericardium or a pericardial effusion before myocardial dysfunction occurs.

• Journal of Chest Surgery
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• 제27권7호
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• pp.617-620
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• 1994

Approximately 10% of patients who have undergone atrial repair for transposition of the great arteries display easily identifiable RV dysfunction with or without TV incompetence by 10years. Treatment is difficult & the surgical options for this complication are limited. We experienced two cases of the reversal of atrial repair and conversion to an arterial switch after initial pulmonary artery banding. This surgical program is a realistic modality treating late failure of systemic right ventricle after atrial switch operation.

• Journal of Genetic Medicine
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• 제11권1호
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• pp.11-15
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• 2014

Congenital central hypoventilation syndrome (CCHS) is a disorder of the autonomic nervous system characterized by a decreased response to hypercarbia. CCHS is frequently associated with congenital megacolon; the combination is called Haddad syndrome. CCHS is associated with dysfunction in respiratory features of the autonomic nervous system and with other disorders, including facial deformities, cardiovascular symptoms, and tumors. Patients with CCHS frequently have a mutation in the homeobox protein 2b (PHOX2B) gene. Most mutations involve heterozygous expansion of alanine repeats (GCN). Interestingly, a higher polyalanine repeat number is associated with a more severe clinical phenotype. To clarify the role of PHOX2B in disease pathogenesis, we introduce and review the clinical and molecular features of CCHS and Haddad syndrome.

• Journal of Chest Surgery
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• 제33권10호
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• pp.830-833
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• 2000

Homograft has been the conduit of choice in various types of congenital malformations which require right ventricular outflow tract reconstruction. However it has been proven to be less than ideal in young age group because of early failure of the conduite due to valve dysfunction and calcification. Furthermore limitation of availability of homograft particularly small sized conduits for neonates and infants is the most serious problem. A 19 month old female patient with pulmonary atresia and ventricular septal defect was operated on with a bovine jugular venous valved conduit as an alternative to the homograft for her right ventricular outflow tract reconstruction. Postoperative hemodynamic performance of the conduit was excellent without pressure gradient or valve regurgitation. With this early result bovine jugular venous valved conduit seems to be another excellent conduit because of good hemodynamics and size availability but long term follow up is necessary.

• 수면정신생리
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• 제3권2호
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• pp.77-89
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• 1996

Data collected to date indicate that sleep apnea syndrome is a risk factor in the development of cardiovascular dysfunction such as systemic hypertension, cardiac arrhythmias, pulmonary hypertension, right heart failure, angina, myocardial infarction, and stroke. Several reports suggest that untreated patients with sleep apnea syndrome have a higher long-term mortality than treated patients and than normal subjects. But there are many controversies concerning the treatment of sleep apnea syndrome, and there is no universally accepted standard for effective treatment. We reviewed the current approaches and options in the medical treatment of sleep apnea syndrome.

• Journal of Yeungnam Medical Science
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• 제27권1호
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• pp.52-56
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• 2010

Idiopathic peripartum cardiomyopathy is an uncommon malady disease. Making the diagnosis is often difficult and it is always necessary to exclude other prior heart disease and other causes of left ventricular dysfunction in pregnant women. Heart failure in these women ensues when the cardiovascular demands of normal pregnancy are further amplified when the common complications of pregnancy complications superimposed upon these underlying conditions that cause compensated ventricular hypertrophy. This may be aggravated by making a late diagnosis and providing inappropriate treatment. We experienced a 38-year-primigravida who has diagnosed with idiopathic peripartum cardiomyopathy and underwent elective cesarean section with general anesthesia.

• Clinical and Experimental Pediatrics
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• 제57권1호
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• pp.1-18
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• 2014

Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.