• Journal of Chest Surgery
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• 제24권7호
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• pp.719-724
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• 1991

A case of pulmonary atresia with intact ventricular septum was presented in a 10-month-old cyanotic female patient, which was congenitally rare. Infant with pulmonary atresia and intact ventricular septum usually require urgent surgical intervention. Angiogram showed the pulmonary atresia at the level of the pulmonary valve, the hypoplasia of tricuspid valve and atrial septal defect without patent ductus arteriosus. We performed the pericardial patch graft on the right ventricular outflow tract and pulmonary artery after ventriculotomy using pacemaker wire as electrical saw and main pulmonary arteriotomy and then modified Waterston shunt from the ascending aorta to patch on the right ventricular outflow tract without extracorporeal circulation. Patient was postoperatively good condition.

• Journal of Chest Surgery
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• 제20권2호
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• pp.432-437
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• 1987

Pulmonary atresia with intact ventricular septum is a extremely rare congenital cardiac anomaly. With the history of cyanosis and failure to thrive, this anomaly should not be excluded and emergency management is necessary. Our patient was 69 day-old male with pulmonary atresia which was confirmed by cardiac angiography. Prostaglandin E1 was used for maintenance of pulmonary blood flow preoperatively. Right ventricular outflow reconstruction with pericardial patch and concomitant pulmonary valvotomy were done on beating heart for palliation. With this method, growing of right ventricle and tricuspid annulus are highly expected.

• Journal of Chest Surgery
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• 제19권3호
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• pp.464-469
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• 1986

Pulmonary atresia with intact ventricular septum, and with VSD were uncommon congenital anomalies with high mortality in the neonatal period. Those survivals depend on an adequate interatrial communication or interventricular communication and pulmonary flow via large aortopulmonary collateral including PDA. Recently we experienced surgical correction of 2 cases pulmonary atresia with intact ventricular septum and with VSD. On case 1, 10-years old male patient was confirmed as pulmonary valvular membranous atresia with intact ventricular septum combined with large functioning PFO and mild tricuspid incompetence. So we performed total correction under the E.C.C, that was PDA ligation, RVOT reconstruction with monocusp valved outflow patch [16mm], repair of tricuspid insufficiency and closure of PFO. Post-operative hemodynamic result was good and there was no event during hospital course. On case 2, 16-years old female patient was diagnosed as pulmonary atresia with VSD and PDA. MPA was absent, remained fibrous cord like remnant and type of VSD was subaortic defect [3cm by 3cm in the size]. PDA was located at the usual site. Under the E.C.C. VSD patch closure through the right ventriculotomy, anastomosis between the right ventricular outflow tract and the pulmonary bifurcated site with the extra-cardiac Hancock valved conduit [22cm] and PDA ligation were performed.

• Journal of Chest Surgery
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• 제56권4호
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• pp.290-293
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• 2023

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital heart disease that often needs a critical decision on whether to open the right ventricular outflow tract (RVOT). Significant morbidity and considerable mortality might preclude the safe use of percutaneous or surgical right ventricular decompression in patients with muscular PAIVS. We report the case of a 21-day-old neonate weighing less than 3 kg who underwent hybrid RVOT stent insertion as initial palliation for muscular PAIVS and subsequent anatomical correction at 5 months of age, with 6 years of follow-up.

• Journal of Chest Surgery
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• 제22권5호
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• pp.766-774
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• 1989

Four neonates with critical pulmonary outflow obstruction underwent emergency palliative operation between February 1988 and May 1989 at the department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital. There were three boys and one girl, the mean age was 12.25 days [ranging from 3 days to 26 days], and the mean body weight was 3,625 gm [ranging from 3,450 gm to 4,200 gm]. Two patients had severe pulmonic valvular stenosis with intact ventricular septum, a third had pulmonary atresia with intact ventricular septum, and another had pulmonary atresia with ventricular septal defect. All were cyanotic, showed arterial desaturation with metabolic acidosis, and had congestive heart failure. To relieve the pulmonary outflow tract obstruction, we performed transventricular pulmonary valvulotomy [Brock operation] with a mosquito clamp in all cases without extracorporeal circulation. Three patients survived the operation and showed satisfactory postoperative results. The patient who had pulmonary atresia with ventricular septal defect expired 5 days after surgery. We consider transventricular pulmonary valvulotomy [Brock operation] with a mosquito clamp as one of the preferable procedures for critical pulmonary outflow obstruction in the neonatal period.

• Journal of Chest Surgery
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• 제22권2호
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• pp.348-356
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• 1989

Seventeen patients of pulmonary atresia with intact ventricular septum were underwent operation during 4.8years period from Jan. 1983 to Aug. 1988 at Seoul National university Hospital. The patients were composed of 8 males and 9 females, aging 1day to 2.5 years [mean 88 days]. We classified pulmonary atresia according to right ventricular morphology; those with tripartite ventricles in 12, those with no trabecular portion to the cavity in 0, and those with inlet portion only in 5. The tripartite approach to right ventricular morphology is helpful in selecting the type of initial palliative procedures. Palliative procedures were as follows; pulmonary valvotomy in 5 with 3 early survivors, mod B-T shunt in 4 with 3 early survivors, and palliative right ventricular outflow tract reconstruction in 4 with 1 early survivor. Effective preliminary palliation of pulmonary atresia are pulmonary valvotomy or palliative right ventricular outflow tract reconstruction in those with tripartite right ventricle, and modified Blalock-Taussig shunt in those with no infundibular portion. The approach to definitive repair is based primarily on the actual size of the tricuspid annulus and the right ventricular cavity. Definitive repair was as follows: definitive right ventricular outflow tract reconstruction in 4 with all survivors and mod. Fontan operation in 2 with one survivors. Right ventricular outflow tract reconstruction can be done as complete repair for patients who had adequate tricuspid annulus and right ventricular cavitary size and mod. Fontan operation for patients who severely hypoplastic tricuspid valve annulus or small right ventricular cavity.

• Clinical and Experimental Pediatrics
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• 제48권11호
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• pp.1256-1256
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• 2005

심실중격결손을 동반하지 않은 폐동맥 폐쇄(pulmonary atresia with intact ventricular septum)는 전체 선천적 심장병의 1%를 차지하는 드문 질환으로 형태학적 특성에 따라 다양한 치료적 접근이 필요하다. 이에 저자들은 정상 크기발달의 우심실을 가진 심실중격결손을 동반하지 않은 폐동맥 폐쇄가 있는 신생아에서 풍선판막성형술 후 지속적인 저산소증을 보여 동맥관 스텐트를 이용하여 거의 정상적인 심장구조와 기능으로 호전된 1례를 경험하였기에 보고하는 바이다.

• 대한흉부심장혈관외과학회:학술대회논문집
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• 대한흉부외과학회 1995년도 제27차년차학술대회 및 총회
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• pp.8-8
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• 1995

• Journal of Yeungnam Medical Science
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• 제7권1호
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• pp.11-18
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• 1990

• Journal of Chest Surgery
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• 제38권6호
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• pp.403-409
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• 2005

온전한 심실중격을 가진 폐동맥 폐쇄증은 병변의 다양성으로 인하여 여러 가지의 외과적 술식이 요구되는 질환이다. 본 연구에서는 온전한 심실중격을 가진 폐동맥 폐쇄증으로 수술을 받은 환자들을 대상으로 수술 방법에 따른 결과 및 장기 성적에 대하여 알아보고자 하였다. 대상 및 방법: 1992년 1월부터 2004년 6월 사이에 외과적 치료를 받았던 총 38명의 환자들을 대상으로 하였다. 평균 연령은 18일$(2\~382일)$이었으며, 삼첨판막륜 크기의 평균 Z값은 $-3.1(-5.6\~0.8)$이었다. 우심실-관상동맥루를 동반한 환자는 13명$(36\%)$이었으며, 우심실의존 관상동맥순환을 보인 환자는 4명$(11\%)$이었다. 평균 추적기간은 55개월(3개월-12.2년)이었다. 결과: 24명의 환자들에서 우심실 감압술이 시행되었고, 14명의 환자들에서는 체폐동맥 단락술만이 시행되었다. 우심실 감압술을 시행받은 환자들의 평균 삼첨판막륜 Z-값은 $-2.2\pm1.1$로 체폐동맥 단락술만 시행받은 환자들의 평균 Z값 $-4.8\pm0.6$보다 컸다(p=0.000). 5명$(13\%)$의 조기 사망과 1명의 만기 사망이 발생하였다. 조기 사망은 우심실 감압술을 시행받은 환자들 중 3명$(13\%)$, 체페동맥 단락술만 시행받은 환자들 중에서 2명$(14\%)$이 발생하였다(p=1.0). 양심실 교정을 받은 환자가 12명$(32\%)$, 단심실 교정을 받은 환자가 8명$(21\%)$, 부분양심실 교정을 받은 환자가 4명$(11\%)$, 최종 교정을 기다리고 있는 환자가 9명$(24\%)$이었다. Kaplan-Meier방법으로 산출한 5년 및 8년 생존율은 $83.2\%$이었다. 결론: 대부분의 사망은 초기 수술 후에 발생하였으며, 양심실 혹은 단심실 교정을 의도한 군간에 사망률의 차이는 없었다. 전체적인 장기 생존율은 비교적 양호하였다. 환자 각각의 삼첨판막륜 및 우심실 크기, 그리고 관상동맥 이상 유무를 정확히 평가하여 적절한 수술 방법을 적용하면 조기 사망률의 개선을 기대할 수 있으리라 생각한다.