• Title/Summary/Keyword: Hypoplastic Left Heart Syndrome

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Modified Norwood Procedure without Circulatory Arrest and Myocardial Ischemia - Report of 2 cases - (완전순환정지와 심근허혈 없이 시행한 변형 Norwood 술식 - 2 례 보고 -)

  • 백만종;김웅한;전양빈;김수철;공준혁;류재욱;오삼세;나찬영;김양민
    • Journal of Chest Surgery
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    • v.34 no.7
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    • pp.547-551
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    • 2001
  • The effects of deep hypothermia and circulatory arrest during aortic arch reconstruction are associated with potential neurologic and myocardial injury. We describe a surgical technique that two patients underwent a modified Norwood procedure without circulatory arrest and myocardial ischemia. One was 13-day-old female patient, weighing 3.1kg, having a variant of hypoplastic left heart syndrome and another was 38-day-old male patient, weighing 3.4 kg, diagnosed Taussig-Bing anomaly with severe aortic arch hypoplasia, coarctation of the aorta, and subaortic stenosis. The arterial cannula was inserted in innominate artery directly. During Norwood reconstruction, regional high-flow perfusion into the inominate artery and coronary perfusion were maintained and there were no neurologic, cardiac, and renal complications in two patients. This technique may help protect the brain and myocardium from ischemic injury in patients with hypoplastic left heart syndrome or other arch anomalies including coarctation or interruption.

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Optimization of the Anastomosis Angle and Diameter with the Systemic- To-Pulmonary Artery Shunt (대동맥-폐동맥 연결관의 접합각도와 직경의 최적화)

  • Kim, Sung-Min;Park, Sung-Yun;Jun, Jae-Hoon
    • Journal of the Korean Society for Precision Engineering
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    • v.24 no.10
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    • pp.123-130
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    • 2007
  • Hypoplastic left heart syndrome is currently the most lethal cardiac malformation of the newborn infant. Survival following a Norwood operation depends on the balance between systemic and pulmonary blood flow, which is highly dependent on the fluid dynamics through the interposition shunt between the two circulations. The purpose of this study is an optimization of the systemic-to-pulmonary artery shunt. In this study, We used computational fluid dynamic(CFD) models to determine the velocity profile in a systemic-to-pulmonary artery shunt and suggested a simplified method of calculating the blood flow in the shunt based on Ultrasound systems. We analyzed the flow characteristic variations and oscillatory shear index(OSI) due to the anastomosis angle and shunt diameter changing. Four different CFD models were constructed with the shunt sizes ranging from 3 to 3.5mm. The angle between the brachiocephalic trunk(BCT) and the shunt were $30^{\circ}$ and $45^{\circ}$, respectively. When the diameter is 3.0 mm, the oscillatory shear index decreased by 1.2% at $30^{\circ}$ as opposed to at $45^{\circ}$. When the diameter is 3.5 mm, it increased by 18% more at $30^{\circ}$ as opposed to at $45^{\circ}$. When the joint angle is $30^{\circ}$ and the diameter is 3.0 mm, the oscillatory shear index decreased by 4.1% in comparison with the 3.5 mm diameter. When the angle is $45^{\circ}$ and the diameter is 3.0 mm, the index increased by 14.6% in comparison with the 3.5 mm diameter.

Emergency Pulmonary Artery-to-Systemic Artery Shunt to Break the Positive Feedback Loop of a Pulmonary Hypertensive Crisis after Neonatal Coarctation Repair

  • Shin, Yu Rim;Yang, Young Ho;Park, Young-Hwan;Park, Han Ki
    • Journal of Chest Surgery
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    • v.52 no.4
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    • pp.232-235
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    • 2019
  • A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.

Recent advances in transcatheter treatment of congenital heart disease (선천성 심질환에 대한 중재적 치료술의 최근 진전)

  • Choi, Jae Young
    • Clinical and Experimental Pediatrics
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    • v.49 no.9
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    • pp.917-929
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    • 2006
  • Over the last several decades there has been a remarkable change in the therapeutic strategy of congenital heart disease. Development of new tools and devices, accumulations of experience, technical refinement have positively affected the outcome of interventional treatment. Many procedures including atrial septostomy, balloon valvuloplasty, balloon dilation of stenotic vessel with or without stent implantation, transcatheter occlusion of abnormal vascular structure, transcatheter closure of patent arterial duct and atrial septal defect, are now performed as routine interventional procedures in many institutes. In diverse conditions, transcatheter techniques also provide complementary and additive role in combination with surgery. Intraoperative stent implantation on stenotic vessels, perventricular device insertion, and hybrid stage 1 palliative procedure for hypoplastic left heart syndrome have been employed in high risk patients for cardiac surgery with encouraging results. Transcatheter closure of ventricular septal defect has been performed safely showing comparable result with surgery. Investigational procedures such as percutaneous valve insertion and valve repair are expected to replace the role of surgery in certain group of patients in the near future. Continuous evolvement in this field will contribute to reduce the risk and suffering from congenital heart disease, while surgery will be still remained as a gold standard for significant portion of congenital heart disease.

Congenital heart disease in the newborn requiring early intervention

  • Yun, Sin-Weon
    • Clinical and Experimental Pediatrics
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    • v.54 no.5
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    • pp.183-191
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    • 2011
  • Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

Norwood Procedure with Home-made Bovine Pericardial Patch (우심낭 조직편을 이용한 Norwood 술식 -2예 보고-)

  • 이석기;김웅한
    • Journal of Chest Surgery
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    • v.36 no.8
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    • pp.602-605
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    • 2003
  • A patch design using bovine pericardial patch for aortic enlargement in the Nowood procedure has been introduced to avoid problems related to homograft availability. We report 2 successful cases of Norwood procedure with home-made bovine pericardial patch. The first case was a 23-day-old (2.2 kg) patient with multi-level left ven-tricular outflow tract obstruction with ductal-dependent systemic circulation. The other case was a 9-day-old (3 kg) patient with hypoplastic lefi heart syndrome. This technique was relatively easy to perform, reproducible and quite effective like homograft patch.

A Clinical Study of Bidirectional Cavopulmonary Shunt (양방향성 상대정맥-폐동맥 단락술의 임상적 연구)

  • 지현근
    • Journal of Chest Surgery
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    • v.28 no.8
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    • pp.759-765
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    • 1995
  • We reviewed our experiences on 33 patients who underwent a bidirectional cavopulmonary shunt[BCPS from February 1992 to July 1994. There were 19 male an 14 female patients, and their weight ranged from 4.4 to 13.3 Kg[mean weight 8.4 $\pm$2.9 Kg . The age ranged from 2 to 55 months [mean age 16.7 $\pm$15.5 months . Their diagnosis included single ventricle group in 16, unbalanced ventricles in 8 whose associated anomalies were double outlet right ventricle, transposition of great arteries and total anomalous pulmonary venous return, tricuspid atresia in 7, hypoplastic left heart syndrome in 1 who underwent a Norwood procedure and double outlet right ventricle with pulmonic stenosis and tricuspid stenosis in 1 who underwent biventricular repair. Among them 10 patients had received other palliative operation before [Norwood procedure 1, pulmonary artery banding 3, modified Blalock-Taussig shunt 6 . The BCPS operations were performed under the cardiopulmonary bypass. 16 patients underwent unilateral BCPS and 17 patients who had bilateral SVC underwent bilateral BCPS. Three patients whose associated anomalies were interruption of IVC underwent total cavopulmonary shunt. There were 5 operative deaths [mortality rate 15.1 % and 2 late deaths. The risk factor for the operation was high mean pulmonary artery pressure [p value<0.05 . The survivors showed good postoperative course and their postoperative oxygen saturation was increased significantly compared to that of preoperative status[p value<0.05 .Conclusively, BCPS operation is effective and safe palliative procedure for the many cyanotic complex congenital anomalies with decreased pulmonary blood flow especialy for the patients who have the high risk factors for Fontan operations.

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Surgical Outcomes of Cor Triatriatum Sinister: A Single-Center Experience

  • Kim, Donghee;Kwon, Bo Sang;Kim, Dong-Hee;Choi, Eun Seok;Yun, Tae-Jin;Park, Chun Soo
    • Journal of Chest Surgery
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    • v.55 no.2
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    • pp.151-157
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    • 2022
  • Background: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS). Methods: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed. Results: The median age and body weight at operation were 9 months (interquartile range [IQR], 3-238 months) and 7.5 kg (IQR, 5.8-49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39-195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6-112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg). Conclusion: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

Complete Transposition of Great Arteries Combined with VSD and Pulmonic Stenosis (S.D.D.) -One Case Report- (대혈관전위증 (S.D.D.) 치험 1례)

  • 강면식
    • Journal of Chest Surgery
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    • v.12 no.3
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    • pp.207-214
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    • 1979
  • This 3-year-old girl was observed frequent exertional dyspnea and cyanosis at crying since birth. She was not premature baby and delivered at full term normally. On physical examination, she was underdeveloped-body weight 13.5 kg, height 99 cm.- and cyanotic. There was severe clubbing on fingers. There was grade II/VI ejection systolic murmur on left lateral border of the sternum. The preoperative examinations [EKG, echocardiogram, cardiac catheterization and biventriculogram] showed that complicated T.G.A. combined vena cava[S.D.D.]. Preoperatively, we decided the corrective surgery of Rastelli operation using a. pulmonary valved conduit. The operation was performed under total circulatory arrest using deep profound hypothermia combining with extracorporeal circulation. On operation, the anatomy of the heart showed that, 1. The subaortic conus was seen and subaortic muscles were hypertrophied. 2. The VSD[type II], behind the subaortic conus-about 1 cm. in diameter, was visible only through LV cavity and, 3. The pulmonary valve ring was hypoplastic and pulmonary valvular stenosis was seen also. The subpulmonic area [LV outflow tract] was obstructed with hypertrophied muscle and mitral valve. 4. Left superior vena cava was drained to RA via coronary sinus. 5. LAD coronary artery was originated from right coronary artery and ran anterior to the pulmonary artery. According to above anatomy, we performed the VSD closure with Teflon patch, and Mustard operation combined with LV-to-pulmonary artery bypass graft using the valve contained [Hancock 16 mm] conduit. Postoperatively, adequate blood pressure could be maintained under the state of using inotropic agent [epinephrine]. On the second postoperative day, the patient died of cardiac arrest due to low cardiac output syndrome, acute renal failure and pulmonary edema.

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Outcome of the Modified Norwood Procedure: 7 Years of Experience from a Single Institution (변형된 노우드(Norwood) 수술의 결과: 단일병원의 지난 7년간의 경험)

  • Kim, Hyung-Tae;Sung, Si-Chan;Kim, Si-Ho;Bae, Mi-Ju;Lee, Hyoung-Doo;Park, Ji-Ae;Chang, Yun-Hee
    • Journal of Chest Surgery
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    • v.43 no.4
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    • pp.364-374
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    • 2010
  • Background: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. Material and Method: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, $11.7{\pm}13.2days$) and weight at operation ranged from 2.2 to 4.8 kg (mean, $3.17{\pm}0.52kg$). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group I) and RMBTS in 8 (group II). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean $26.0{\pm}22.8months$). Result: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. Conclusion: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.