Partial cardiopulmonary bypass through left thoracotomy for coarctation repair in children.

BACKGROUND: A left thoracotomy approach is anatomically appropriate for childhood aortic coarctation; however, the pediatric femoral arteriovenous diameters are too small for cardiopulmonary bypass cannulation. We aimed to determine the safety of a partial cardiopulmonary bypass through the main pulmonary artery and the descending aorta in pediatric aortic coarctation repair. METHODS: We retrospectively reviewed 10 patients who underwent coarctation repair under partial main pulmonary artery-to-descending aorta cardiopulmonary bypass with a left thoracotomy as the CPB group. During the same period, 16 cases of simple coarctation of the aorta repair, with end-to-end anastomosis through a left thoracotomy without partial CPB assistance, were included as the non-CPB group to evaluate the impact of partial CPB. RESULTS: The median age and weight at surgery of the CPB group were 3.1 years (range, 9 days to 17.9 years) and 14.0 (range, 2.8-40.7) kg, respectively. Indications for the partial cardiopulmonary bypass with overlap were as follows: age > 1 year (n = 7), mild aortic coarctation (n = 3), and predicted ischemic time > 30 min (n = 5). Coarctation repair using autologous tissue was performed in seven cases and graft replacement in three. The mean partial cardiopulmonary bypass time, descending aortic clamp time, and cardiopulmonary bypass flow rate were 73 ± 37 min, 57 ± 27 min, and 1.6 ± 0.2 L/min/m2, respectively. Urine output during descending aortic clamping was observed in most cases in the CPB group (mean: 9.1 ± 7.9 mL/kg/h), and the total intraoperative urine output was 3.2 ± 2.7 mL/kg/h and 1.2 ± 1.5 mL/kg/h in the CPB and non-CPB group, respectively (p = 0.020). The median ventilation time was 1 day (range, 0-15), and the intensive care unit stay duration was 4 days (range, 1-16) with no surgical deaths. No major complications, including paraplegia or recurrent coarctation, occurred postoperatively during a median observation period of 8.1 (range, 3.4-17.5) years in the CPB group. In contrast, reoperation with recurrent coarctation was observed in 2 cases in the non-CPB group (p = 0.37). CONCLUSIONS: Partial cardiopulmonary bypass through the main pulmonary artery and descending aorta via a left thoracotomy is a safe and useful option for aortic coarctation repair in children.

[Stanford Type A Acute Aortic Dissection with Myonephropathic Mrtabolic Syndrome].

A 57-year-old man was transferred with sudden onset chest pain and evolving paralysis and numbness in the left leg. Contrast computed tomography (CT) revealed Stanford type A acute aortic dissection from the ascending aorta to bilateral internal and external iliac arteries with blood flow obstruction to the left kidney and left lower limb. Surgery was initiated 10 hours after onset of ischemic symptoms in the leg. Femoro-femoral bypass was carried out first, and we ensured sufficient phlebotomy from the ischemic limb during reperfusion and continuous hemodiafiltration to prevent myonephropathic metabolic syndrome. Total aortic arch replacement was then performed. Our treatment strategy was effective in this case of Stanford type A aortic dissection with prolonged lower limb ischemia. Although left hip disarticulation was subsequently required due to intractable infection, the patient became able to walk with an artificial limb after post-rehabilitation.

Surgical repair of the adult form of scimitar syndrome.

Scimitar syndrome has various clinical presentations and anatomic features, and some cases are diagnosed in adulthood. Reconstruction using autologous tissue to reroute the right pulmonary venous blood flow to the left atrium is ideal. However, if the scimitar vein drains to the caudal segment of the inferior vena cava, reconstruction using prosthetic material may be necessary due to the distance between the left atrium and the scimitar vein. We describe the case of a 16-year-old boy with scimitar syndrome. We anastomosed the scimitar vein to the right atrium using an artificial graft and created an atrial septal defect for rerouting the right pulmonary venous blood to the left atrium. It has been 9 years since this procedure, and the patient has not experienced graft stenosis or thromboembolic events.

Fetal cardiac capillary hemangioma resulting in tachyarrhythmia.

We present the case of a fetus with cardiac capillary hemangioma in the right atrial cavity. The tumor showed dramatic growth between the 28th and 32nd week of gestation and resulted in tachyarrhythmia. The patient was born at the 33 weeks of gestation weighing 2430 g via urgent cesarean section because the rapidly growing cardiac tumor caused incessant tachyarrhythmia, pericardial effusion, and fetal circulatory incompetence. Coronary angiography revealed that the right coronary artery drained into the tumor. Due to hemodynamic deterioration, the patient underwent subtotal resection of the tumor on the 2nd day after birth. Histopathological examination revealed an undifferentiated capillary hemangioma. The patient was discharged at the age of 86 days, as the tachyarrhythmia and hemodynamic incompetence had subsided; however, bradycardia and intermittent atrioventricular conduction disturbance gradually developed. Capillary hemangioma, a rare primary cardiac space-occupying tumor in children, can invade the conduction system.

Postoperative Maximal Aortic Diameter is a Significant Predictor of Dilation of the Residual Dissected Aorta after Aortic Replacement for Acute Debakey Type I Aortic Dissection.

BACKGROUND: This study investigated the impact of aortic diameter on late aortic dilation of the residual dissected aorta after tear-oriented aortic replacement for acute DeBakey type I aortic dissection. METHODS: Of 133 patients who underwent aortic replacement for acute DeBakey type I/II aortic dissection between 2008 and 2019, 45 patients with a residual dissected aorta after surgery for acute DeBakey type I aortic dissection and who underwent computed tomography at predischarge and after 1 year were retrospectively assessed. The aortic diameter and false lumen area were measured at 3 levels: the maximal aortic site, seventh thoracic vertebra, and celiac axis. Multivariable Cox regression analysis was employed to identify the predictors of late aortic dilation, defined as an aortic growth rate of ≥5 mm/year or a maximal aortic diameter of ≥55 mm. RESULTS: During a median follow-up of 75 [range: 13-152] months, 6 patients (5 men; mean age: 57 ± 14 years) experienced aortic dilation. All 6 patients had the maximal aortic diameter between the distal aortic arch and seventh thoracic vertebra level at the last computed tomography. Multivariable Cox regression analysis showed that the predischarge maximal aortic diameter was an independent determinant of late aortic dilation (hazard ratio: 2.28/mm, 95% confidence interval: 1.10-5.86). CONCLUSIONS: Predischarge maximal aortic diameter is a significant predictor of late aortic dilation in patients with a residual dissected aorta after tear-oriented surgical repair of acute DeBakey type I aortic dissection.

Coronary artery patch augmentation for congenital left coronary ostial stenosis in Williams syndrome.

Left coronary ostial stenosis, which is associated with sudden death, occasionally occurs in individuals with Williams syndrome. However, surgical methods that provide reliable long-term revascularization remain unknown among infants and young children with coronary ostial stenosis. We describe the case of an 18-month-old boy with Williams syndrome who presented with cardiogenic shock due to left coronary ostial stenosis. We performed patch augmentation of the left coronary ostium using glutaraldehyde-treated autologous pericardium. At the last follow-up, the patient was well without any adverse events or myocardial ischemia.

Unilateral Embolization of the Internal Iliac Artery for Endovascular Aortic Repair Does Not Induce Gluteal Muscle Atrophy.

BACKGROUND: To investigate the effect of unilateral internal iliac artery (IIA) embolization for endovascular aortic repair (EVAR) on gluteal muscle size. METHODS: We assessed the gluteal muscle size in 111 consecutive patients who underwent elective EVAR with unilateral IIA embolization (n = 31) or without IIA embolization (n = 80) for abdominal aortic and/or iliac artery aneurysm. The cross-sectional area (CSA) of the gluteus maximus (Gmax) and gluteus medius/minimus (Gmed/min) was measured on computed tomography preoperatively, 6 months postoperatively, and final follow-up. Mean changes in the Gmax and Gmed/min CSA were evaluated using a mixed model analysis of variance. RESULTS: In the patients with embolization, both the Gmax and Gmed/min CSA significantly decreased over time on the embolization and nonembolization sides (P < 0.001); however, embolization did not affect the changes in the Gmax CSA (P = 0.64) and Gmed/min CSA (P = 0.99). In the patients with embolization and those without embolization, both the Gmax and Gmed/min CSA significantly decreased over time (P < 0.001); however, embolization did not affect the changes in the Gmax CSA (P = 0.76) and Gmed/min CSA (P = 0.11). CONCLUSIONS: Unilateral IIA embolization was not associated with gluteal muscle atrophy after EVAR. Pre-emptive unilateral IIA embolization for EVAR seems to be an acceptable procedure in terms of maintenance of gluteal muscle size.

Bilateral Coronary-Pulmonary Artery Fistulas in Pulmonary Atresia With Ventricular Septal Defect.

We present a very rare case of bilateral coronary to pulmonary artery fistulas associated with pulmonary atresia with ventricular septal defect. The courses of coronary to pulmonary artery fistulas have to be clearly delineated by detailed angiography prior to corrective surgery.

Resection of Kommerell Diverticulum After the Arterial Switch for TGA With Bilateral PDAs and Right Aortic Arch.

We present a very rare case of bilateral ductus arteriosus in transposition of the great arteries with right aortic arch and aberrant retroesophageal left subclavian artery (SCA). Around 1 month after the successful arterial switch operation, the baby showed wheezing and retractive breathing. The computed tomography revealed that trachea and esophagus were sandwiched between the posterior displaced ascending aorta and the origin of the retroesophageal aberrant left SCA, the so-called Kommerell diverticulum (KD). This compression was successfully relieved by resection of the KD and division of the retroesophageal aberrant SCA through right thoracotomy.

Relationship between pulmonary arterial resistance and compliance among patients with pulmonary arterial hypertension and congenital heart disease.

BACKGROUND: It is unknown whether changes in pulmonary arterial resistance (Rp), pulmonary arterial compliance (Cp), and the product of Rp and Cp (the RC time) in patients with pulmonary arterial hypertension (PAH) are related to an increase in pulmonary blood flow. The aim of this study is to clarify relationships between these parameters before and after corrective surgery among patients with PAH and congenital heart disease. METHODS: We performed cardiac catheter examinations and compared Rp, Cp, and the RC time before and after corrective surgery in 100 infants (53 boys) with PAH related to ventricular septal defect. RESULTS: Median age at surgery was 2.9 (0.6-28.5) months. Preoperative values of the ratio of pulmonary to systemic blood flow (Qp/Qs), systolic pulmonary arterial pressure (PAP), Rp, Cp, and the RC time were 3.5 (2.7-4.3), 65 (56-70) mm Hg, 2.18 (1.64-3.19) Wood unit/m(2), 2.67 (2.01-3.38) mL/mm Hg/m(2), and 0.36 (0.31-0.40) seconds, respectively. Postoperative systolic PAP, Cp, and the RC time were significantly decreased to 26 (23-29) mm Hg, 1.96 (1.77-2.26) mL/mm Hg/m(2), and 0.31 (0.26-0.36) seconds, respectively, although Rp remained unchanged at 2.53 (2.06-3.31) Wood unit/m(2). The relationship between Rp and Cp was inversely related before and after surgery, and the Rp-Cp coupling curve was shifted downward after surgery. In addition, lower preoperative Cp (partial regression coefficient = -3.35; P = .001) and preterm delivery (partial regression coefficient = 6.28; P = .02) were independently related to postoperative higher systolic PAP. CONCLUSIONS: Rp-Cp coupling depends on both the amount of pulmonary blood flow and the condition of the pulmonary vasculature. Lower preoperative Cp is an independent predictive factor to predict higher postoperative pulmonary systolic pressure leading to an increase in right ventricular workload. It is necessary to assess Rp-Cp coupling before surgery among patients with PAH related to congenital heart disease.

Tricuspid regurgitation resulting from acute type A aortic dissection.

Proximal extension of acute type A aortic dissection can affect the aortic valve but seldom affects the tricuspid valve. We report the case of an octogenarian who underwent successful surgical repair of an aortic dissection that was accompanied by tricuspid regurgitation. We believe that the tricuspid regurgitation was attributable to displacement of the valve resulting from aortic dissection.

Early and Long-term Effects of the Autologous Peripheral Stem Cell Implantation for Critical Limb Ischemia.

OBJECTIVE: Therapeutic angiogenesis by peripheral blood mononuclear cells (PB-MNCs) implantation has been shown to be a safe and effective treating for critical limb ischemia (CLI). We herein report our investigation of the long-term efficacy of implantation of granulocyte-colony stimulating factor (G-CSF)-induced PB-MNCs to treat patients with CLI for which surgical bypass and/or percutaneous transluminal angioplasty are not possible. Methods and Methods : Eleven cases were enrolled in this study. Following an injection of G-CSF (250 ug/day) for 3 days, PB-MNCs (1.1 ± 0.5 × 10(10) including 1.5 ± 0.2 × 10(7) CD34-positive cells) were harvested by apheresis and then injected into 13 ischemic limbs. RESULTS: Resting pain either diminished or improved in 10 cases (91%) at 4 weeks, and ulcer formation was cured in 6 out of 10 limbs (60%) after treatment. The time required to enhance the arteries at the level of foot-joint by angiography which was performed in the abdominal aorta was shortened by 1 month (10 ± 4 seconds) and 6 months (12 ± 1) compared with the pretreatment time (15 ± 5). Three patients died after treatment, and the actuarial survival rate at 3 years was 73%. Freedom from major amputation at 3 years was 92%. CONCLUSION: The local injection of G-CSF-induced PB-MNCs showed striking early and long-term effects.

Early results of bilateral pulmonary artery banding for hypoplastic left heart syndrome.

OBJECTIVE: To compare the haemodynamics and perioperative course of initial palliation with bilateral pulmonary artery banding (PAB) and the Norwood procedure. METHODS: Between April 2004 and December 2007, 43 consecutive children with hypoplastic left heart syndrome (HLHS) or a variant underwent initial palliation (PAB, n=18; Norwood, n=25). Clinical perioperative data were analysed. In the PAB group, lipo-prostaglandin E1 administration was continued with hospitalisation until stage 2 palliation with a bi-directional Glenn shunt and the Norwood procedure. RESULTS: There were no significant differences in the age and operative weight of patients who received stage 1 palliation (PAB, 12+/-9 days, 2.7+/-0.6 kg; Norwood, 12+/-8 days, 2.8+/-0.4 kg). The PAB group had more high-risk patients than the Norwood group (PAB, 83%; Norwood, 48%, p=0.04). Increased early and inter-stage mortality were observed in patients who underwent the Norwood procedure (early mortality with PAB, 6% vs Norwood, 12%; inter-stage mortality, 6% vs 27%, respectively). Mortality between stages 1 and 2 was 11% for the PAB group and 36% for the Norwood group. The Kaplan-Meier survival estimate at 1 year did not differ between groups (77% for the PAB group, 64% for the Norwood group). Ductal stenosis was found in one patient in the PAB group during the follow-up period. Twenty-eight patients underwent stage 2 reconstruction, and the patients in the PAB group were younger at the time of surgery (PAB, 116 days; Norwood, 224 days). There were no significant differences between groups in pulmonary artery index regarding body surface area (BSA) (PAB, 179 mm(2)BSA(-1); Norwood, 194 mm(2)BSA(-1)) and the incidence of ventricular dysfunction after stage 2 construction (PAB, 21%; Norwood, 21%). CONCLUSIONS: Bilateral PAB with continuous lipo-prostaglandin E1 administration may improve early and intermediate mortality in infants with HLHS. Intimate care with hospitalisation may contribute to the results.