• Title/Summary/Keyword: 폐동맥판협착

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Corrective Surgery of Congenital Cardiac Anomalies in the Noonan syndrome - Report of two cases - (Noonan 증후군에 동반된 심기형의 수술적 교정 - 2례 보고 -)

  • 이선희;이주현;심성보;박재길;곽문섭;김세화;오용석;윤호중;정욱성
    • Journal of Chest Surgery
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    • v.34 no.7
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    • pp.552-555
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    • 2001
  • Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovate. The second 28-year-old male patient had valvular and subvalvular Pulmonary stenosis with typical Noonan\`s face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovate was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

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Congenital Quadricuspid Semilunar Valve - A case report - (선천성 사엽성 반월형 판막 - 1예 보고 -)

  • Seo, Min-Bum;Seo, Hong-Joo
    • Journal of Chest Surgery
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    • v.42 no.3
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    • pp.361-363
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    • 2009
  • A 17-year-old male patient was referred with symptoms of dyspnea. Multi-detector computerized tomography (MDCT) and echocardiography evaluation revealed quadricuspid aortic and pulmonary valves, an atrial septal defect (ASD), and pulmonary stenosis. We closed the ASD using a bovine patch and performed a commissurotomy of the pulmonary valve. Quadricuspid semilunar valves are very rare congenital abnormalities that are reported to occur nine times more frequently in the pulmonic valve than in the aortic valve. According to the Hurwitz Roberts classification, the aortic valve was type A, and the pulmonic valve was type B. The aortic valve normal function, but the pulmonic valve was stenotic and had abnormal function.

Aortic Valve Replacement with Pulmonary Autograft in Patient with Congenital Aortic Stenosis : Ross Procedure without Homograft -one case report - (선천성 대동맥판 협착증에서 폐동맥판 자가이식편을 이용한 대동맥판 교체술:동종판막을 쓰지 않는 Ross술식)

  • 이은상;윤태진;서동만
    • Journal of Chest Surgery
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    • v.32 no.3
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    • pp.303-306
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    • 1999
  • This is a genuine case report of the Ross operation without the use of homografts or heterografts in reconstruction of the right ventricular outflow tract. A 8-year-old boy with congenital aortic stenosis underwent aortic valve replacement with a pulmonary autograft and right ventricular outflow tract reconstruction with a pericardial conduit bearing autologous aortic monocusp. The postoperative echocardiography and cardiac angiography revealed good ventricular function and competent neoaortic valve. He has been followed up for 19 months.

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Pulmonary Valve Replacement with Tissue Valves After Pulmonary Outflow Tract Repair in Children (소아에서 폐동맥유출로 재건 후 시행한 조직판막을 이용한 폐동맥판 대치술)

  • Lee, Jeong-Ryul;Hwang, Ho-Young;Chang, Ji-Min;Lee, Cheul;Choi, Jae-Sung;Kim, Yong-Jin;Rho, Joon-Ryang;Bae, Eun-Jung
    • Journal of Chest Surgery
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    • v.35 no.5
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    • pp.350-355
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    • 2002
  • Background: Most of pulmonary regurgitation with or without stenosis appears to be well tolerated early after the repair of pulmonary outflow tract. However, it may result in symptomatic right ventricular dilatation, dysfunction and arrhythmias over a long period of time. We studied the early outcome of pulmonary valve replacement with tissue valves for patients with the above clinical features. Material and Method: Sixteen consecutive patients who underwent pulmonary valve replacement from September 1999 to February 2002 were reviewed(9 males and 7 females). The initial diagnoses included tetralogy of Fallot(n=11), and other congenital heart anomalies with pulmonary outflow obstruction(n=5). Carpentier-Edwards PERIMOUNT Pericardial Bioprostheses and Hancock porcine valves were used. The posterior two thirds of the bioprosthetic rim was placed on the native pulmonary valve annulus and the anterior one third was covered with a bovine pericardial patch. Preoperative pulmonary regurgitation was greater than moderate degree in 13 patients. Three patients had severe pulmonary stenosis. Tricuspid regurgitation was present in 12 patients. Result: Follow-up was complete with a mean duration of 15.8 $\pm$ 8.5months. There was no operative mortality. Cardiothoracic ratio was decreased from 66.0 $\pm$ 6.5% to 57.6 $\pm$ 4.5%(n=16, p=0.001). All patients remained in NYHA class I at the most recent follow-up (n=16, p=0.016). Pulmonary regurgitation was mild or absent in all patients. Tricuspid regurgitation was less than trivial in all patients. Conclusion: In this study we demonstrated that early pulmonary valve replacement for the residual pulmonary regurgitation with or without right ventricular dysfunction was a reasonal option. This technique led to reduce the heart size, decrease pulmonary regurgitation and tricuspid regurgitation as well as to improve the patients'functional status. However, a long term outcome should be cautiously investigated.

One-stage Repair of Interrupted Aortic Arch with Ventricular Septal Defect and Valvular Aortic Stenosis - A case report- (심실 중격 결손과 대동맥 판 협착을 가진 대동맥 궁 단절의 일차 완전 교정술 -1예 보고-)

  • Cho, Joon-Yong;Jeong, Young-Kyun;Lee, Jong-Tae;Kim, Kyu-Tae;Chang, Bong-Hyun
    • Journal of Chest Surgery
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    • v.38 no.12 s.257
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    • pp.856-859
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    • 2005
  • A male neonate was referred to our hospital with facial cyanosis and tachypnea at 19 days of age. Two-dimensional echocardiography showed type B interrupted aortic arch, posterior malalignment ventricular septal defect and valvular aortic stenosis. A new surgical repair was done with biventricular repair and neo-aortic arch reconstruction. Left ventricular outflow track (LVOT) was consisted of aortic valve and pulmonic valve. Right ventricular outflow (RVOT) track was reconstructed with extracardiac conduit. Postoperative two-dimensional echocardiography showed no stenosis and turbulency flow on LVOT and RVOT.

Cardiac Lymphoma in a Shih Tzu Dog (씨츄 개에서 발생한 심장 림프종)

  • Kim, Tae-Young;Joo, Jeong-Hoon;Hyun, Changbaig
    • Journal of Veterinary Clinics
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    • v.30 no.3
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    • pp.196-200
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    • 2013
  • A 7 years-old intact female dog (4.8 kg) was referred with primary complaints of enlarged surface lymph nodes, cardiac murmur, coughing and exercise intolerance. Diagnostic imaging studies found cardiomegaly with distended caudal vena cava, marked left ventricular and interventrciular septal thickening and flattening, severe aortic and pulmonic stenosis (~5 m/s), and mitral and tricuspid regurgitation (~4 m/s). Cytology for the samples obtained from submandibular lymph node and left ventricle revealed high grade malignant lymphoma. The case was diagnosed as cardiac lymphoma. The dog was treated with prednisolone (2 mg/kg, PO, q24h), lomustine (80 $mg/m^2$ PO, q3wk), diltiazem (1 mg/kg, PO, q12h) and enalalpril (0.5 mg/kg, PO, q12h). The clinical signs were improved after therapy. The dog is still on the chemotherapy and regularly checked up.

Surgical Correction of Tetralogy of Fallot in Adults over 20 Years of Age (20세이상 성인 팔로사징후의 완전교정술에 관한 임상적 고찰)

  • 류완준;강종렬;조창욱;김정철;구본일;이홍섭;김창호;우건화;이신영
    • Journal of Chest Surgery
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    • v.29 no.11
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    • pp.1197-1201
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    • 1996
  • From October, 1987 to December, 1995, nine patients underwent total correction of tetralogy of Fallot in adults over 20 years of age. There were 5 male and 4 female patients aged from 22 to 42 years(mean, 29.6 years). Three patients were in New York Heart Association(NYHA) functional class II, and 6 patients in class III. The hemoglobin values ranged from 10.8 to 20.7 gm/㎗ (average, l5.6gm/㎗). The preoperative clinical features were as follows: cyanosis, 8 patients; dyspnea on exertion, 6 patients; clubbing of fingers, 5 patients; frequent upper respiratory infection, 3 patients. At the operation both infundibular and valvular stenosis were present in all patients. Reconstruction of right ventricular outflow tract(RVOT) using Goretex was required in 7 patients, and transannular patching with Goretex in 2 pateints. Left pulmonary angioplasty with pericardium was done in 2 patients. No hospital deaths occurred. Four of 9 patients(44.4%) had postoperative low cardiac output syndrome, and postoperative bleeding in 5. One patient required reoperation due to residual ventricular septal defect and tricuspid regurgitation 3 months after the first operation. The mean follow-up period was 25 months, range 11 to 77 months. All was asymptomatic and in NYHA class I. We suggest that advanced age is not contraindication to surgery in tetralogy of Fallot, and tetralogy of Fallot in adults could be operated on due to low mortality.

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Arterial Switch Operation for Transposition of G rest Arteries (대혈관전위증에 대한 동맥전환술)

  • 이호철;류한영
    • Journal of Chest Surgery
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    • v.29 no.3
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    • pp.278-284
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    • 1996
  • Nine infants with transposition of great arteries have undergone arterial switch operation from May 1989 to May 1994 in the Department of Thoracic and Cardiovascular Surgery, Yeungnam University Hospital. Patients' age ranged from 3 days to 90 days, averaging 30$\pm$21 days. Diagnosis was made by two-dimensional echocardiography in all patients. Eight patients were diagnosed as transposition of great arteries with ventricular septal defect and one patient was a simple transposition of great arteries. Associated anomalies were patent ductus arteriosus (8), atrial septal defect (7) and coarctation of aorta(1). The anatomy of the coronary arteries were 7 (77 %) type A and 2 (23 %) type D according to the Yacoub classification. Pulmonary artery reconstruction was done according to Lecompte maneuver with tautologous pericardial patch in 8 patients. Overall operative mortality rate was 55% Left heart failure and pulmonary hypertensive crisis were the cause of death on postoperative 1~2 days in three patients, and two succumbed to death due to sepsis on postoperative 2~ 3 weeks. The mean follow-up period was mean 17 months. No patient had clinically significant postoperative aortic regurgitation and supravalvular pulmonary stenosis. The excessive use of inotropic support postoperatively was identified as a stastically significant risk factor following the arterial switch operation. But other variables such as low body weight, long cardiopulmonary bypass time, excessive hemodilution during cardiopulmonary bypass, hypothermia and volume loading were not significant risk factors.

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Surgical Treatment of Atrial Septal Defect in Adult - Clinical Review of 31 Cases - (성인의 선천성 심방중격결손증의 외과적 치료)

  • 장운하;오태윤;배상일
    • Journal of Chest Surgery
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    • v.31 no.8
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    • pp.770-775
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    • 1998
  • Background: Atrial septal defect (ASD) is the most common congenital cardiac anomaly, accounting for 30 percent of congenital heart disease detected in the adult. Many patients with ASD are well tolerated and reach adult without significant symptoms. The patients with ASD die 4th and 5th decades, but prolonged survival is not uncommon. In general, the survival depends on whether pulmonary hypertension develops during adulthood or not. The most common cause of death in the patients with ASD is right ventricular failure or arrhythmias. Materials and methods: From January 1988 to June 1997, 33 cases of ASD underwent open heart surgeries in our hospital. Among them, 31 cases were adult ASD, and 2 tricuspid regurgitation, 1 pulmonic stenosis, 1 mitral regurgitation, 1 tricuspid regurgitation, and 1 coronary artery disease were combinded. All of the patients underwent surgical repair using autologus pericardial patch or direct closure. Results: The postoperative course was smooth and uneventful. Most of the patients showed significant improvement in ECG finding, hemodynamic profile, radiologic finding, and echocardiography, after surgery. Conclusions: Conclusively, most of the ASD should be closed even in patients over the age of 60 years, and early surgical repair must be done to prevent pulmonary hypertension, right ventricular failure, and arrythmias.

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Early Failure of the Shelhigh Pulmonary Valve Conduit in Ross Operation - Two case reports- (Ross 수술시 사용한 Shelhigh 폐동맥판 도관의 조기 실패 -2예 보고-)

  • Jang Woo Sung;Kim Dong Jung;Kim Jin Hyun;Han Kook Nam;Choi Chang Hyu;Kim Woong Han
    • Journal of Chest Surgery
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    • v.38 no.5 s.250
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    • pp.382-384
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    • 2005
  • Background: It is very difficult to choose the ideal valved conduit used in right ventricle outflow reconstruction in child. We can use the cryopreserved homograft but there is a limit of application because of its difficulties in the size matching and supply capacity. The $Shelhigh^{(R)}$porcine-valved conduit is commercially available and used as an alternative choice in these days. We report two cases of early Shelhigh conduit failure in right ventricular outflow tract after Ross operation in congenital aortic stenosis.