• Journal of Chest Surgery
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• v.34 no.9
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• pp.720-723
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• 2001

A case of Marfan’s syndrome with atresia of right coronary artery is reported. A 45-year-old woman, who was diagnosed as Marfan’s syndrome 1 year ago, came to the hospital complaining of acute chest pain. The patient showed arachynodactyly, pectus carinatum, and long and slender extremities. In echocardiography there were severe aortic regurgitation measured grade IV and aortic dilatation of ascending aorta maximally 5.9 cm in diameter. Mitral regurgitation was mild, but there were also moderate left ventricular dilation and moderately decreased ejection fraction of left ventricle. At operation, atresia of right coronary artery was found. We performed Bentall type operation with SJM 27mm valved conduit for left coronary artery, and Piehler’s modification for right coronary artery bypass using 6mm PTFE graft. The atretic portion of right coronary artery from the suspected right coronary ostium to distal coronary flow was about 4 cm in length. The combination of right coronary artery atresia and Marfan’s syndrome is very rare. The author describes the rare case, which is treated with combined technique of Bentall and Piehler modification for reconstruction of coronary circulation.

• Journal of Chest Surgery
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• v.54 no.1
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• pp.72-74
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• 2021

Congenital atresia of the left main coronary artery (LMCA) is an extremely rare coronary anomaly that necessitates surgical correction. Patients with LMCA atresia may have various clinical symptoms, which are determined by the degree of collateral vessel development from the right coronary system, the metabolic demands of the heart, and concomitant mitral insufficiency caused by myocardial ischemia. Unlike in adults, there are limited surgical options for coronary artery disease in children. Herein, we report a case of LMCA atresia with mitral regurgitation in a 19-month-old child that was successfully corrected by coronary artery bypass grafting and mitral valve repair.

• Journal of Chest Surgery
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• v.39 no.10 s.267
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• pp.779-781
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• 2006

Left main coronary artery atresia is a very rare congenital coronary anomaly with blind end of left main trunk. The clinical symptoms as syncope, failure to thrive, and myocardial infarction are presented and surgical treatments are required in most cases. We report a case of a 14-months-old girl with left main coronary artery atresia and excel-lent surgical result of 1 year follow-up after coronary artery bypass with left internal thoracic artery.

• Journal of Chest Surgery
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• v.15 no.4
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• pp.394-401
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• 1982

Recently, 4 cases underwent successful surgical correction of tetralogy of Fallot combined with pulmonary atresia in 2 cases, with abnormal coronary artery in another 2 cases. The operation consisted of a patch repair to the ventricular septal defect. The pulmonary atresia and stenosis were corrected with the method of external connection, from the right ventricle to the pulmonary artery using the valved conduits.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.290-300
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• 2000

Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.

• Journal of Chest Surgery
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• v.38 no.6 s.251
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• pp.403-409
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• 2005

Pulmonary atresia with intact ventricular septum is morphologically heterogeneous, and the surgical outcome remains suboptimal compared to other complex congenital heart defects. We evaluated the long-term results for repair of pulmonary atresia with intact ventricular septum, Material and Method: Between January 1992 and June 2004, 38 patients underwent repair of pulmonary atresia with intact ventricular septum. The average age was 18 days $(2\~382\;days)$. The average Z-value of the tricuspid annulus diameter was -3.1$(-5.6\~0.8)$. Thirteen $(36\%)$ patients had right ventricle-to-coronary artery fistulas, and $4(11\%)$ patients had right ventricle-dependent coronary circulation. Average follow-up was 55 months $(3\;months\~2.2\;years)$, Result: Twenty-four patients under-went initial right ventricle (RV) decompression and 14 patients underwent systemic-to-pulmonary arterial shunt only. The average size of the tricuspid annulus of the patients who underwent RV decompression was significantly larger than that of the patients who underwent systemic-to-pulmonary arierial shunt only (Z-value -2.2 vs. -4.8, p=0.000). There were $5(13\%)$ early and 1 late deaths. Early deaths occurred in 3 patients who had undergone RV decompression, and in 2 patients who had undergone systemic-to-pulmonary arterial shunt only (p=1.0). Biventricular repair was achieved in $12(32\%)$ patients, single ventricular repair in $8(21\%)$, and one and a half ventricular repair in $4(l1\%)$ patients. Nine $(24\%)$ patients are waiting for the definitive repair. Kaplan-Meier survival at 5 and 8 years was $83.2\%$, respectively. Conclusion: Most of the deaths occurred after the initial palliation. Overall long-term survival was satisfactory. Early mortality should be reduced with careful preoperative evaluation and proper surgical strategy.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.594-600
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• 1989

Primary intracardiac repair of tetralogy of Fallot with low mortality and early good results, has been accomplished in recent years. But palliative procedures have been reserved for those hypoplastic pulmonary arteries, a hypoplastic left ventricle or anomalies of the coronary artery would make total correction difficult. And the Blalock-Taussig shunt operation is recognized as a standard and popular palliative procedure. I undertook a retrospective determination of the effect of the Blalock-Taussig shunt operation on the development of the main pulmonary artery and the right and left pulmonary arteries. Between January, 1980, and April, 1987, at the Severance Hospital, 16 patients were studied by cardiac catheterization and angiocardiography, before undergoing Blalock-Taussig shunting procedures for the palliation of severe symptoms of tetralogy of Fallot, and some time later, usually prior to a second procedure. The mean interval between catheterizations was 22.25 months. Patients with tetralogy of Fallot and pulmonary atresia or with an occluded shunt were not included. The primary and secondary angiograms of each patient were reviewed, and measurements of the diameter of the main pulmonary artery, the right and left pulmonary arteries, and the descending thoracic aorta were taken. The results are as follows; 1. The hematocrit decreased from 56.39% to 50.34%[p< 0.05], and the arterial oxygen saturation increased from 62.00 % to 81.31 %[p< 0.001] following shunt procedures 2. The ratio of the diameter of the right pulmonary artery plus the left pulmonary artery to the diameter of the descending thoracic aorta increased 1.30 k 0.28 times [p< 0.01]; but the ratio of the diameter of the main pulmonary artery to the diameter of the descending thoracic aorta increased 1.10 * 0.33 times, which was not. significant[p< 0.05]. 3. The interval between shunting and second catheterization was not related to the magnitude of change in the pulmonary arteries[r=0.141, p >0.05]. 4. The changes in the ratio of the diameter of the right pulmonary artery plus the diameter of the left pulmonary artery to the diameter of the descending thoracic aorta was inversely related to the initial ratio[r=0.757, p >0.001], but the change in the ratio of the diameter of the main pulmonary artery to the descending thoracic aorta was not related[r=0.059, p >0.05]. 5. There were no differences in enlargement of the pulmonary artery on the side of the shunt [ipsilateral] versus enlargement on the opposite side [p >0.05], nor according to the size of the shunt[p >0.05]. In conclusion, this study suggests that the Blalock-Taussig shunt is effective for the development of the right and left pulmonary arteries but not effective for the main pulmonary artery.

• Journal of Chest Surgery
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• v.29 no.10
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• pp.1081-1089
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• 1996

Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality and morbidity. This mAy attribute to the non-uniformity of the anomaly. We reviewed a total of 34 infants with pulmonary atresla and intact ventricular septum managed in this hospital between 1987 and 1995. Mean age and body weight were 57.2 (range, 3-208) days and 4.1 (range, B.3∼6.8) kg. The preoperative Z-value of the diameter of the tricuspid valve was less than -2 in 85.2% of patients and less than -4 in 33. 3% . It is well correlated w th right ventricular cavity size (n=27. r10.68, p< 0.05). Coronary artery-right ventricular fistulas were identified in 3 patients, and right ventricular dependency was suspected in 1 Over All hospital mortality was 23.5%(8/34), although it decreased to 16.6%(4124) in 1990s. Subsequent procedures were performed in 6 patients between 3 days and 58 months after Initial palliation : one bidirectional cavopulmonary shunt and 1 Fontan operation after systemic-pulmonary shunt, 3 transannular patch + atrial septal defect closure and 1 additional systemic-pulmonary shunt after polmonary valvectomy or valvotomy. Changes of Z-values of the diameter of tricuspid valve have been followed up in 11 patients between 1 and 66 months postoperatively. Z-values were increased In 5 out of 8 transannular right ventriculAr outflow tract enlargement group and in 1 out of 3 pulmonary valvectomy or valvotomy group. Our data suggest that tailoring a treatment to right ventricular cavity size and coronary anom lies may improve the surgical outcome. A Z-value of the tricuspid valve diameter could be used.

• 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.