Background: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half ($1\text\tiny{1/2}$) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. Methods: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or $1\text\tiny{1/2}$ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3-24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. Results: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54-68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62-128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. Conclusion: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or $1\text\tiny{1/2}$ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.
Takayasu's arteritis(aortoarteritis) is a chronic inflammatory disease involving the aorta, the arteries arising from the aorta, and the pulmonary arteries also may be involved. The inflammation leads to either stenosis and occlusion of the involved artery or formation of aneurysm or both. The arterial lesions can lead to secondary hypertension, retinopathy, cardiac involvement, cerebrovascular events, and premature death. The course and prognosis of patients with aortoarteritis show wide variation, and few authors have published systematic studies documenting the natural history of this disease. While the etiology of Takayasu's arteritis remains unknown, various modes of treatment including steroids, vascular surgery, and balloon angioplasty have been used for management of these patients. We experienced a case of Takayasu's arteritis with multiple occlusion of pulmonary arteries, which was confirmed by angiography and perfusion scan, so reported it with a review of literature.
Kim, Hyung-Tae;Sung, Si-Chan;Chang, Yun-Hee;Jung, Won-Kil;Lee, Hyoung-Doo;Park, Ji-Ae;Huh, Up
Journal of Chest Surgery
/
v.44
no.6
/
pp.392-398
/
2011
Background: The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. Materials and Methods: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was $72{\pm}37$ months (range: 4~160 months). Results: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. Conclusion: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.
Background: Pulmonary artery banding(PAB) accompanies some risks in the aspect of band complications and mortality in the second-stage operation. To assess these risks of the second-stage operation after PAB, we reviewed the surgical results of the second-stage operation in the pediatric patients who had undergone PAB in infancy. Material and Method: From May 1988 to June 1997, a total of 29 patients with preliminary PAB underwent open heart surgery. Ages ranged from 2 to 45 months(mean 20.6$\pm$9.0 months). Preoperative congestive heart failure conditions were improved after PAB(elective operation group) in 27 patients, but early second-stage procedures were required in the remaining 2 patients due to sustaining congestive heart failure(early operation group). Preoperative surgical indications included 2 double outlet right ventricles(DORV group) and 27 ventricular septal defects as the main cardiac anomaly(VSD group). Result: The mean time interval from PAB to the second-stage operation was 15.5$\pm$8.7 months(range 5 days to 45 months). One patient in the DORV group underwent intraventricular tunnel repair and modified Glenn procedure in the other. In the VSD group, the VSD was closed with a Dacron patch in all patients. Concomitant procedures included a right ventricular infundibulectomy in 4 patients and a valvectomy of the dysplastic pulmonary valve in 1 patient. At the second-stage operations, pulmonary angioplasty was required due to the stenotic banding sites in 18 patients. One patient underwent complete ligation of the main pulmonary artery with the modified Glenn procedure. The mortality at the second-stage operation was 17.2%(5 patients). Causes of death were 4 low cardiac output, and 1 autoimmune hemolytic anemia. Diagnosis with DORV and the early operative group were the risk factors for operative death in this series. There was 1 late death. Conclusion: This study revealed the second-stage operation for pulmonary artery debanding and closure of VSD in children was complicated by the correction of the acquired lesions with a significantly high incidence of morbidity and early postoperative deaths. Primary repair is recommended for isolated VSD, if possible.
Objective: To quantitatively assess the pulmonary vasculature using non-contrast computed tomography (CT) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) pre- and post-treatment and correlate CT-based parameters with right heart catheterization (RHC) hemodynamic and clinical parameters. Materials and Methods: A total of 30 patients with CTEPH (mean age, 57.9 years; 53% female) who received multimodal treatment, including riociguat for ≥ 16 weeks with or without balloon pulmonary angioplasty and underwent both non-contrast CT for pulmonary vasculature analysis and RHC pre- and post-treatment were included. The radiographic analysis included subpleural perfusion parameters, including blood volume in small vessels with a cross-sectional area ≤ 5 mm2 (BV5) and total blood vessel volume (TBV) in the lungs. The RHC parameters included mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), and cardiac index (CI). Clinical parameters included the World Health Organization (WHO) functional class and 6-minute walking distance (6MWD). Results: The number, area, and density of the subpleural small vessels increased after treatment by 35.7% (P < 0.001), 13.3% (P = 0.028), and 39.3% (P < 0.001), respectively. The blood volume shifted from larger to smaller vessels, as indicated by an 11.3% increase in the BV5/TBV ratio (P = 0.042). The BV5/TBV ratio was negatively correlated with PVR (r = -0.26; P = 0.035) and positively correlated with CI (r = 0.33; P = 0.009). The percent change across treatment in the BV5/TBV ratio correlated with the percent change in mPAP (r = -0.56; P = 0.001), PVR (r = -0.64; P < 0.001), and CI (r = 0.28; P = 0.049). Furthermore, the BV5/TBV ratio was inversely associated with the WHO functional classes I-IV (P = 0.004) and positively associated with 6MWD (P = 0.013). Conclusion: Non-contrast CT measures could quantitatively assess changes in the pulmonary vasculature in response to treatment and were correlated with hemodynamic and clinical parameters.
Background and Objectives: The recent developments in chronic thromboembolic pulmonary hypertension (CTEPH) are emphasizing the multidisciplinary team. We report on the changes in clinical practice following the development of a multidisciplinary team, based on our 7 years of experience. Methods: Multidisciplinary team was established in 2015 offering both balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) with technical upgrades by internal and external expertise. For operable cases, PEA was recommended as the primary treatment modality, followed by pulmonary angiography and right heart catheterization after 6 months to evaluate treatment effect and identify patients requiring further BPA. For patients with inoperable anatomy or high surgical risk, BPA was recommended as the initial treatment modality. Patient data and clinical outcomes were closely monitored. Results: The number of CTEPH treatments rapidly increased and postoperative survival improved after team development. Before the team, 38 patients were treated by PEA for 18 years; however, 125 patients were treated by PEA or BPA after the team for 7 years. The number of PEA performed was 64 and that of BPA 342 sessions. World Health Organization functional class I or II was achieved in 93% of patients. The patients treated with PEA was younger, male dominant, higher pulmonary artery pressure, and smaller cardiac index, than BPA-only patients. In-hospital death after PEA was only 1 case and none after BPA. Conclusions: The balanced development of BPA and PEA through a multidisciplinary team approach proved synergistic in increasing the number of actively treated CTEPH patients and improving clinical outcomes.
The optimal surgical management of the coarctation of the aorta associated with ventricular septal defect is still debated. Sixteen patients with the coarctation of the aorta and VSD were operated upon between November, 1980 and September 1984 at Seoul National University Hospital. They were 11 males and 5 females. All presented between 5 months and 11.5 years of age [mean= 5.5 years]. Presenting symptoms were congestive heart failure in 11 [69%], cyanosis on crying in 3 [19%], and frequent upper respiratory infection in 2 [13%]. In all cases two-stage operation was applied except one in which one stage procedure was taken. Resection and end-to-end anastomosis was used in 3, Dacron graft in 5, Gortex graft in 1, and left subclavian flap angioplasty in 4. Remaining two were missed on the operating table before correction of the coarctation of the aorta. Overall operative death in repair of the coarctation of the aorta were 3[20%]. Among the 12 survivors after repair of the coarctation of the aorta, 4 required patch closure of VSD, 2 required primary closure, 2 showed spontaneous closure [17%], one [8%] showed decrease in its size, 3 were under observation. It might be safe to approach the coarctation of aorta plus VSD with initial repair of the coarctation of the aorta without banding of main pulmonary artery and later management of VSD as usual manner in simple VSD.
Background: Tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAS) is complex lesion with marked heterogeneity of pulmonary blood supply and arborization anomalies. Patients with TOF with PA and MAPCAS have traditionally required multiple staged unifocalization of pulmonary blood supply before undergoing complete repair. In this report, we describe recent change of strategy and the results in our institution. Material and Method: We established surgical stratagies: early correction, central mediastinal approach, initial RV-PA conduit interposition, and aggressive intervention. Between July 1998 and August 2004, 23 patients were surgically treated at our institution. We divided them into 3 groups by initial operation method; group I: one stage total correction, group II: RV-PA conduit and unifocalization, group III: RV-PA conduit interposition only. Result: Mean ages at initial operation in each group were $13.9{\pm}16.0$ months (group 1), $10.4{\pm}15.6$ months (group II), and $7.9{\pm}7.7$ months (group III). True pulmonary arteries were not present in f patient and the pulmonary arteries were confluent in 22 patients. The balloon angioplasty was done in average 1.3 times (range: $1{\sim}6$). There were 4 early deaths relating initial operation, and 1 late death due to incracranial hemorrhage after definitive repair. The operative mortalities of initial procedures in each group were 25.0% (1/4: group I), 20.0% (2/10: group II), and 12.2% (1/9: group III). The causes of operative mortality were hypoxia (2), low cardiac output (1) and sudden cardiac arrest (1). Definitive repair rates in each group were 75% (3/4) in group I, 20% (2/10, fenestration: 2) in group II, and 55.0% (5/9, fenestration: 1) in group III. Conclusion: In patients of TOF with PA and MAPCAS, RV-PA connection as a initial procedure could be performed with relatively low risk, and high rate of definitive repair can be obtained in the help of balloon pulmonary angioplasty. One stage RV-PA connection and unifocalization appeared to be successful in selected patients.
The pulmonary giant cell carcinoma is classified as a variant of a large cell carcinoma and is diagnosed by the minimum component of 10% huge, pleomorphic and multinucleated giant tumor cell and emperipolesis of the neutrophils into the tumor cells. This tumor is characterized by local recurrences and early metastasis with extremely short patient survival. However, there are some reports that state that the survival time was extended by the operative resection and postoperative adjuvant chemotherapy and radiotherapy. A 46-year old male was admitted with complaint of hemoptysis for 2 months. Through chest X-ray and chest CT, a 5cm sized mass was found in the apical segment of the right upper lobe. During the preoperative evaluation, stenotic lesion in the left anterior descending coronary artery was found and treated by percutaneous transarterial coronary angioplasty. Four weeks later, right upper lobectomy was performed and the mass was proven to be a giant cell carcinoma. The patient received adjuvant chemotherapy and radiotherapy.
We reviewed our 18-year surgical experience of biventricular repair for double-outlet right ventricle. Material and Method: One hundred twelve consecutive patients (80 males and 32 females) who underwent biventricular repair for double-outlet right ventricle between May 1986 and September 2002 were included. We assessed risk factors for early mortality and reoperation. Reoperation-free survival rate and actual survival rate were analysed. Result: Most common type of ventricular septal defect was subaortic (n=58, 52%) and non-committed type was second most common (n=32, 29%). Four different surgical methods were used: intraventricular baffle repair (n=71 , 63%): right ventricle to pulmonary ariery conduit interposition or REV with left ventricle to aorta baffle repair (n=24, 21 .4%): arierial switch operation with left ventricle to pulmonary artery baffle (n=14, 12.5%): Senning atrial switch operation with left ventricle to pulmonary artery baffle (n=3, 2.7%). Thirty four patients(30%) underwent palliative procedures before definite repair. Twenty three patients (21%) required reoperations. There were 12 (10.7%) early deaths and 4 late deaths. Age younger than 3 months at repair (p=0.003), cardiopulmonary bypass and aortic cross clamp time (p=0.015, p=0.067), type of operation (arterial switch operation) (p <0.001) and type of ventricular septal defect (subpulmonic type) (p=0.002) were revealed as risk factors for early death in univariate analysis, while age under 3 months was the only significant risk factor in multivariate analysis. Patients younger than 1 year of age (p=0.02), pulmonary artery angioplasty at definitive repair (p=0.024), type of ventricular septal defect (non-committed) (p=0.001), type of operation (right ventricle to pulmonary artery conduit interposition and REV operation) (p=0.028, p=0.017) were risk factors for reoperation in univariate analysis but there was no significant risk factor in multivariate analysis. Follow-up was available on 91 survivals with a mean duration of 110.8$\pm$56.4 (2~201) months. 5, 10 and 15 year survival rates were 86.5%, 85% and 85% and reoperation free survival were 85%, 71.5%, 70%. Conclusion: Age under 3 months at repair, subpulmonic ventricular septal defect and arterial switch operation were significant risk factors for early mortality. Patients with non-committed ventricular septal defect and who underwent conduit interposition or REV operation were risk factors for reoperation. With careful attention to chose best timing and surgical approach depending on morphologic characteristics, biventricular repair for double outlet right ventricle can be achieved with good long-term outcome.
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