• Journal of Chest Surgery
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• v.12 no.3
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• pp.281-288
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.289-296
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.727-733
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• 1988

Two cases of the discrete membranous subaortic stenosis were experienced at the Department of Thoracic & Cardiovascular Surgery, National Medical Center, Seoul, Korea. Case I was 31 years old male with a history of aortic valve replacement[Ionescu-Shiley, 19mm] at the other hospital in 1980. Heart failure was noticed 6 years later. On cardiac catheterization, pressure gradient between left ventricle 4 aorta was 104 mmHg, but subvalvular aortic stenosis was not detected by preoperative 2-D echo <% left ventriculogram. Above case revealed stenoinsufficiency of the prosthetic valve. Under the diagnosis of the prosthetic valve failure, re-do aortic valve replacement[Bjork-Shiley, 23 mm] was done. During operation, the discrete membranous subaortic stenosis was incidentally found, and it was completely resected. So we thought that above discrete membranous subaortic stenosis was not detected at first operation, and it was progressed during 6 years, and accelerated the degeneration of the prosthetic valve. Case II was 20 years old female. Her complaints were exertional dyspnea, angina, syncope, which were aggravated since 5 years ago. 2-D echo <% left ventriculogram revealed the discrete membranous subaortic stenosis. Pressure gradient was 20 mmHg, but her symptoms were serious. Associated cardiac anomaly was the persistent left superior vena cava without connection with right superior vena cava. Complete excision of the membranous tissue was done. Post-operative pressure gradient between left ventricle & aorta was absent, and her complaints were nearly subsided. Both cases were type I according to the Newfeld classification of the discrete subvalvular aortic stenosis, and complete excision of the membranous tissue was done without myotomy or mymectomy. And short-term follow-up results[Case I:2 years, Case II: 1 ~ years] were good except soft systolic murmur[grade II/VI] at the aortic area in both cases.

• Journal of Chest Surgery
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• v.20 no.3
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• pp.536-543
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• 1987

The Fontan procedure was physiological correction which was initially applied to tricuspid atresia. We had used the modified Fontan operation in 8 cases at National Medical Center, Seoul, from Aug. 1984 to Oct. 1986. Age range was 20 months to 15 years [mean: 9 years] and male: female ratio was 5:3. 5 patients had tricuspid atresia [lb: 2 cases, Ic: 1 case, lib: 1 case, & llc: 1 case], 2 Patients had univentricular heart of left ventricular type, and one patient had transposition of great arteries with complete endocardial cushion defect. The operative principle was direct anastomosis between right atrium and pulmonary artery, whether main pulmonary artery or right pulmonary artery without any conduits. Postoperatively all patients needed high central venous pressure for adequate hemodynamic status in both survival [20-24 CmH2O] and mortality groups [20-24 CmH2O]. The fatal complications were as follows: empyema with bronchopleural fistula [1 case], bleeding tendency & brain damage [1 case], low cardiac output syndrome & acute renal failure [2 cases], and right to left shunt of unknown origin [1 case]. There were 5 hospital deaths; 3 of 5 tricuspid atresia patients, 1 of 2 univentricular heart patients, and 1 of 1 transposition of great arteries with complete endocar4ial cushion defect patient. The overall mortality was 62.5%. 3 survivors were nearly compatible with Choussat & Fontan criteria. Thus appropriate patient selection and experienced surgical technique were required for good results.

• Archives of Plastic Surgery
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• v.49 no.3
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• pp.369-372
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• 2022

Reduction mammaplasty is the gold standard treatment for gigantomastia. We report one female patient with juvenile gigantomastia associated with severe pulmonary hypertension where her pulmonary pressure decreased significantly after the surgery, improving her quality of life. A 22-year-old female patient with gigantomastia since 10 years old, tricuspid regurgitation, and pulmonary thromboembolism antecedent was admitted to the emergency department. Her oxygen saturation was 89%. Acute heart failure management was initiated. An echocardiogram reported left ventricle ejection fraction (LVEF) of 70% with severe right heart dilation, contractile dysfunction, and arterial pulmonary pressure (PASP) of 110 mm Hg. A multidisciplinary team considered gigantomastia could generate a restrictive pattern, so a Thorek reduction mammoplasty with Wise pattern was performed. Presurgical measurements were: sternal notch to nipple-areola complex, right 59 cm, left 56 cm. Three days after surgery, the patient could breathe without oxygen support. In the outpatient follow-up, patient referred reduction of her respiratory symptoms and marked improvement in her quality of life. Six months after surgery, a control echocardiogram showed a LVEF of 62% and PASP of 85 mm Hg. Pulmonary hypertension may be present in patients with gigantomastia. Reduction mammoplasty may be a feasible alternative to improve the cardiac signs and symptoms in patients with medical refractory management.

• Journal of Cardiovascular Imaging
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• v.31 no.4
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• pp.169-177
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• 2023

BACKGROUND: Acute worsening of cardiac function frequently leads to kidney dysfunction. This study aimed to identify clinical and imaging parameters associated with impaired kidney function in patients with acute decompensated heart failure with reduced ejection fraction (HFrEF). METHODS: Data from 131 patients hospitalized with acute decompensated HFrEF (left ventricular ejection fraction, < 40%) were analyzed. Patients were divided into two groups according to the glomerular filtration rate (GFR) at admission (those with preserved kidney function [GFR ≥ 60 mL/min/1.73 m²] and those with reduced kidney function [GFR < 60 mL/min/1.73 m²]). Various echocardiographic parameters and perirenal fat thicknesses were assessed by computed tomography. RESULTS: There were 71 patients with preserved kidney function and 60 patients with reduced kidney function. Increased age (odds ratio [OR], 1.07; 95% confidence interval [CI], 1.04-1.12; p = 0.005), increased log N-terminal pro b-type natriuretic peptide (OR, 1.74; 95% CI, 1.14-2.66; p = 0.010), and increased perirenal fat thickness (OR, 1.19; 95% CI, 1.10-1.29; p < 0.001) were independently associated with reduced kidney function, even after adjusting for variable clinical and echocardiographic parameters. The optimal average perirenal fat thickness cut-off value of > 12 mm had a sensitivity of 55% and specificity of 83% for kidney dysfunction prediction. CONCLUSIONS: Thick perirenal fat was independently associated with impaired kidney function in patients hospitalized for acute decompensated HFrEF. Measurement of perirenal fat thickness may be a promising imaging marker for the detection of HFrEF patients who are more susceptible to kidney dysfunction.

• Journal of Chest Surgery
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• v.57 no.3
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• pp.312-314
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• 2024

A 3-year-old boy with Glenn physiology exhibited refractory heart failure with reduced ejection fraction. To improve the patient's oxygen saturation, he underwent ventricular assist device (VAD) implantation with concomitant Fontan completion. The extracardiac conduit Fontan operation was performed with a 4-mm fenestration. For VAD implantation, Berlin Heart cannulas were positioned at the left ventricular apex and the neo-aorta. Following weaning from cardiopulmonary bypass, a temporary continuous-flow VAD, equipped with an oxygenator, was utilized for support. After a stabilization period of 1 week, the continuous-flow VAD was replaced with a durable pulsatile-flow device. Following 3 months of support, the patient underwent transplantation without complications. The completion of the Fontan procedure at the time of VAD implantation, along with the use of a temporary continuous-flow device with an oxygenator, may aid in stabilizing postoperative hemodynamics. This approach could contribute to a safe transition to a durable pulsatile VAD in patients with Glenn physiology.

• Journal of Veterinary Clinics
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• v.41 no.2
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• pp.106-111
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• 2024

A 4-year-old neutered female domestic shorthair cat weighing 5.1 kg was referred to Jeju National University Hospital with acute onset respiratory distress, weakness, and anorexia. The patient had a history of stressful antecedent events that involved bullying by a newly introduced cat. Thoracic radiography and echocardiography revealed a stage C hypertrophic cardiomyopathy phenotype based on the American College of Veterinary Internal Medicine classification system with pulmonary edema, pleural effusion, and pericardial effusion at the same time. The patient was treated with furosemide, pimobendan, and rivaroxaban. Pericardiocentesis was performed because pericardial effusion was identified. Reevaluation after 30 days revealed a normal respiratory rate on physical examination, normal cardiac shape on thoracic radiographs, and normal cardiac measurements on echocardiography. The patient was tentatively diagnosed with transient myocardial thickening (TMT) and all medications were discontinued. Six months after the initial hospitalization, the cat continued to do well without any clinical signs or left ventricular wall thickening. This case is the first report describing feline TMT in Korea. Moreover, it involves a rare case in which pulmonary edema, pleural effusion, and pericardial effusion, which induce cardiac tamponade, occurred simultaneously due to TMT-related congestive heart failure.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.247-252
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• 1997

Ten acute and chronic in vivo studies were utilized to develop a pneumatic ventricular assist device (VAD) as a bridge to heart transplantation or a circulatory assist device for patients with end-stage heart disease or poor myocardial funct on after cardiac surgery. Two sizes of blood pump of 70cc for adult patients and 34cc for pediatric patients were implanted in the animals. Ventricle of the blood pump was made from the polyurethaae to enhance antithrombogenecity. The VAD was implanted between the left atrium and the descending aorta. Average flow rate was 2.38 L/min for adult and 0.41 L/min for pediatric VAD at the rate of 60 bum. The duration of support ranged from 1 to 26 hours. The most frequent complication was bleeding. Main causes of death were heart failure and respiratory failure. The device function was good for short term use. Studies to date suggest that, with further refinement, a reliable long term VAD that will have clinical application can be developed.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.129-132
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• 2009

We describe here our experience with a neonate presenting with cyanosis, grunting, and cardiomegaly, who was diagnosed with isolated left ventricular noncompaction (IVNC) by echocardiography. The patient had high levels of N-terminal pro-B-type natriuretic peptide (NT pro-BNP) and symptoms of heart failure including poor feeding and tachypnea. During the period in which NT pro-BNP levels steadily increased, the patient suffered sudden cardiac arrest despite heart failure management. Following cardiopulmonary resuscitation, cardiac arrest was resolved, NT pro-BNP levels decreased, and all symptoms showed improvement. We consider that assessment of NT pro-BNP with cardiac functional analysis using echocardiography could help in the prediction of disease progress in IVNC.