Management of Multiple Renal Arteries and Unusual Venous Anatomy During Kidney Transplant: From a Simple Technical Problem to a Graft-Saving Procedure.

OBJECTIVES: Incidence of vascular anomalies in donor kidneys varies from 18% to 30% and presents a challenge for a transplant surgeon in kidney transplant. Here we present our personal experience for man - agement of the complicated and unexpected cases. MATERIALS AND METHODS: A total of 250 kidney transplants (226 living, 24 deceased) were performed in a period of 24 years; mean donor age was 55 years (range, 25-86 years), and mean recipient age was 38.6 years (range, 14-66 years). We analyzed the surgical techniques, complications and outcomes, rejection episodes, kidney function, and graft and patient survival rates. RESULTS: Of 250 nephrectomies, 209 had a single artery (83.6%), 34 had 2 arteries (13.6%), and 7 had 3 arteries (2.8%). Of 34 double arteries, 14 had 2 main arteries, 15 had a main and a polar artery, and 5 had an aortic Carrel patch after deceased donation. According to the size, type, and position, the anastomoses were performed with branches of hypogastric, epigastric inferior, iliac external, and main renal artery, intracorporeally or in bench surgery. Regarding veins, 1 double inferior vena cava, 1 left-side inferior vena cava, 4 retroaortic, 2 circumaortic, 10 large lumbar veins draining into the left renal veins, and 8 cases with 2 or more different size renal veins were managed. In 9 cases with short right renal vein, an extension with vena cava (a "Barry cavoplasty") was performed in deceased donor organs. No serious surgical complications related to vascular anomalies were observed. There were no statistical differences in 1-, 6-, and 12-month graft survival rates between the groups with or without vascular anomalies. CONCLUSIONS: Vascular anomalies should no longer be considered a contraindication for transplant, if careful anastomosis is performed in every case to avoid ischemia and further complications. Therefore, management of vascular anomalies could be a graftsaving procedure.

Furcate insertion of the umbilical cord: pathological and clinical characteristics in 132 cases.

Objectives Furcate cord insertion is a rare abnormality affecting approximately 0.1% of all pregnancies. Macroscopically, the umbilical vessels separate before reaching the placenta, lose their Wharton's jelly, and insert at the placenta centrally, eccentrically, or marginally. The aim of this retrospective study was to determine the prevalence of furcate cord insertion more accurately, the pathological characteristics, and clinical outcomes. Methods We conducted a retrospective study of 132 cases of furcate insertion of the umbilical cord using the pathological database of the Charité University Hospital Berlin, Germany, between 1993 and 2016. This included 99 cases, including one termination of pregnancy within our institution and 33 cases from external hospitals. An analysis of the pathological features of the 132 cases and the perinatal outcome of the 98 cases within our institution were performed. Results Furcate cord insertion occurred in 0.16% pregnancies. Of the 132 cases, seven cases of intrauterine fetal deaths were observed. Three of those could be linked to the furcate cord insertion. In two of those cases, single umbilical vessel rupture was identified as the cause of fetal death. Conclusions In most cases of furcate cord insertion, the outcome is good; however, intrauterine fetal death occurs in approximately 1.02% of cases.

Repair of pulmonary artery sling with tracheal and intracardiac defects.

BACKGROUND: Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions. METHODS: Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3-9). Surgical approaches included pulmonary artery sling alone (n = 10), pulmonary artery sling with tracheoplasty (n = 41), and pulmonary artery sling with both intracardiac and tracheal surgery (n = 28). RESULTS: There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2-24.8) and 17.9 (interquartile range 4.3-19.8) days, and considerably longer when associated tracheal surgery (p = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality. CONCLUSION: Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.

Selective Antegrade Cerebral Perfusion With or Without Additional Lower Body Perfusion During Aortic Arch Reconstruction in Infants.

BACKGROUND: Aortic arch reconstruction is often challenging, especially in infants, owing to its high postoperative complication risks. This study aimed to compare the effectiveness between selective antegrade cerebral perfusion (SACP) alone and SACP in combination with continuous lower body perfusion with descending aortic cannulation (DAC) in preserving renal function, and to determine the influence of perfusion strategy on the postoperative course of infants who underwent aortic arch reconstruction. MATERIAL AND METHODS: A total of 121 infants who underwent aortic arch reconstruction between January 2008 and December 2018 were included in the analysis. Patients (median age: 29 days, range: 3-270 days) were divided into the following groups: those who underwent repair with SACP (SACP group, 79 patients) and those who underwent additional lower body perfusion (DAC group, 42 patients). RESULTS: Three (7.1%) and nine (11.4%) patients died in the DAC and SACP groups, respectively (P = .54). The SACP group had more patients requiring renal replacement therapy (P = .002) and higher incidence of second stage acute kidney injury (AKI) development (Kidney disease improving global outcomes (KDIGO) criteria; P = .032). The SACP group had higher frequency of open chest postoperatively than the DAC group (P = .011). The DAC group had lower vasoactive inotropic score (VIS) at the first postoperative day (P < .001) and shorter intensive care unit length of stay (P = .050). There was no difference in neurological complications between the groups (P = .061). High VIS was associated with early mortality (odds ratio [OR]: 1.79 [1.33-2.41], P < .001) and AKI (OR: 1.60 [1.35-1.91], P < .001). The DAC perfusion strategy with minimal hypothermia was associated with lower risk of AKI (OR: 0.91 [0.84-0.98], P = .016). CONCLUSION: Antegrade cerebral perfusion with continuous lower body perfusion via DAC could effectively be used for improving early postoperative results among infants undergoing procedures that include aortic arch reconstruction.

Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: A Congenital Heart Surgeons' Society study.

OBJECTIVES: We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery. METHODS: We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports. RESULTS: There were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P < .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features. CONCLUSIONS: Anatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.

Retroperitoneal tumors and congenital variations in vascular anatomy of retroperitoneal great vessels.

BACKGROUND/OBJECTIVES: Variations in vascular anatomy (VIVAs) of the retroperitoneal great vessels are uncommon but can potentially complicate surgical procedures and negatively affect treatment outcomes, yet their incidence and clinical impact are poorly studied. We sought to assess the incidence and clinical impact of VIVAs of retroperitoneal great vessels in patients with retroperitoneal tumors. METHODS: We retrospectively analyzed imaging, surgical, treatment and survival data of all pediatric patients with retroperitoneal tumors who underwent resection between January 2007 and October 2016, comparing preoperative scans with corresponding intraoperative observations, and subsequent surgical outcomes. RESULTS: Among 66 children with renal, adrenal and paravertebral tumors, 6 (9%) had retroperitoneal VIVAs. Retroperitoneal VIVAs were present only with right-sided tumors and significantly associated with more frequent intraoperative complications (P = 0.013). While the presence of retroperitoneal VIVAs was not directly associated with survival outcomes, relapse was more frequent in patients with VIVAs (33%) than those without (18.3%, P = 0.378), and relapse was also associated with lower overall and event-free survival (P < 0.001). CONCLUSIONS: VIVAs of retroperitoneal great vessels occurred in 9% of our patients with retroperitoneal tumors. Retroperitoneal VIVAs were associated with higher rates of intraoperative complications and disease relapse but was not directly related to survival outcomes. TYPE OF STUDY: Retrospective review study. LEVEL OF EVIDENCE: Level III Retrospective comparative study.

How Could Aortic Atresia With Interrupted Aortic Arch Survive? About a Neonatal Repair on Two Ventricles.

Aortic atresia with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery. Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an aorto-pulmonary window (AP window), an aberrant right subclavian artery or a bilateral patent ductus arteriosus (PDA). We report a case of aortic atresia with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.

Mortality trends from congenital malformations of the heart and the great vessels in children and adults in the seven socioeconomic regions of Mexico, 2000-2015.

BACKGROUND: Congenital heart disease (CHD) represents a global health problem. In Mexico, in children <1 year of age it is the second cause of mortality. The aim was to determine mortality trends from CHD and the great vessels in children and adults nationwide, by state and socioeconomic region. METHODS: Records of mortality associated to CHD and the great vessels for 2000-2015 were obtained from the National Institute of Statistics and Geography. This information is collected from death certificates issued nationwide. International Classification of Diseases, 10th revision, codes corresponding to the basic cause of death from CHD and the great vessels. To calculate mortality rates for individuals ≥1 year of age, population counts from the national population, estimated by the National Population Council for 2000-2015, were used in the denominator; for infant mortality, live birth counts were used. Rates of mortality nationwide, by state, and by socioeconomic region were calculated. The seven socioeconomic regions were elaborated by the National Institute of Statistics and Geography and include the 31 states and Mexico City according to indicators that are related to well-being such as education, occupation, health, housing, and employment. RESULTS: In the period 2000-2015, 70 741 individuals among children and adults died from CHD. Age-adjusted mortality rate per 100 000 individuals increased from 3.3 to 4. In the age group <1 year of age, mortality rate per 100 000 live births increased from 143.9 to 217.3. CONCLUSIONS: In the period 2000-2015, age-adjusted mortality rate per 100 000 individuals increased from 3.3 to 4. Mexico City as region 7 had the highest mortality from CHD and the great vessels.

Prevalence of Bovine Aortic Arch Variant in Patients with Aortic Dissection and its Implications in the Outcome of Patients with Acute Type B Aortic Dissection.

OBJECTIVE/BACKGROUND: To investigate the prevalence of bovine arch (BA) among patients with type A and B aortic dissection, and to provide insight into the implication of this variation on the outcome of patients with acute or subacute type B aortic dissection (a/sTBAD). METHODS: This retrospective cohort analysis includes patients with a/sTBAD admitted between January 2006 and December 2016. Computed tomographic angiograms (CTAs) of patients referred because of type A aortic dissection were also re-evaluated with regard to the presence of BA. As a control group, 110 oncological patients who had undergone a chest CTA for disease staging during the study period were enrolled. A total of 154 patients with a/sTBAD and 168 with type A aortic dissection were identified during the study period. RESULTS: An overall prevalence of 17.6% for BA variants was revealed. The comparison between patients with aortic dissection and the control group showed no statistically significant difference in BA prevalence (17.7% vs. 17.3%; p = 1.0). No statistically significant difference in BA prevalence was observed when comparing patients with type A aortic dissection with those with type B aortic dissection (16.6% vs. 18.8%; p = .66). During a median follow-up period of 27.8 months, 30 patients died. The mortality rate among patients presenting a BA variant was 34.5%, whereas among patients without, it was 16.0% (p = .04). Multivariate analysis revealed the presence of a BA as an independent predictor of mortality (adjusted odds ratio 3.4, 95% confidence interval 1.2-9.8). CONCLUSION: The BA should be considered as a predictor of the outcome for patients with type B aortic dissection.

Percutaneous cryoablation of symptomatic venous malformations as a second-line therapeutic option: a five-year single institution experience.

PURPOSE: To report the mid-term outcomes of percutaneous cryoablation (PCA) performed as second-line therapeutic option of venous malformations (VM). MATERIAL AND METHODS: From 2011 to 2015, PCA was offered in 24 patients (mean age: 31 years, range: 12-64) as second-line treatment for recurrence of symptoms after sclerotherapy and when resection was not possible (due to lesion location or previous failure) or refused by the patient. Adverse effects were recorded, disease-free survival (DFS) and local tissue control (LTC) rates were calculated based on symptoms and volume evolution. RESULTS: Mean follow-up was 18.7 months (6-48). Nine (37.5%, 9/24) adverse effects occurred and three (12.5%, 3/24) were severe. Mean pain assessed by visual analog scale (VAS) was 41.7 mm (0-80) before treatment and 20.3 mm (0-80) (p=0.01) after. Mean volume decreased significantly after treatment from 22.4 cm3 (0.9-146) to 8.35 cm3 (0-81.3) (p<0.001). Pain recurred in nine patients and size of one lesion increased. The DFS and LTC rates were 54% [95%CI: 22.94-77.27] and 93.33% [61.26-99.03] at 24 months, respectively. Only VM volume >10 cm3 was associated with a higher risk of local recurrence (p=0.05). CONCLUSION: PCA as second-line treatment appears to be safe and effective for local control of VM according to mid-term results. KEY POINTS: • Percutaneous cryoablation of venous malformations appeared well tolerated. • Size of venous malformations decreased significantly after percutaneous cryoablation (p<0.001). • Pain decreased significantly after percutaneous cryoablation of venous malformations (p=0.01).

Surgical treatment of pulmonary atresia with major aortopulmonary collateral arteries in 83 consecutive patients.

OBJECTIVES: This article reports the safety and efficacy of a morphology-based algorithm for the surgical management of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. METHODS: A total of 83 patients were operated from 2007 to 2014 using 3 surgical approaches: one-stage total repair, one-stage unifocalization with central shunt and delayed total repair and multistage unifocalization with subsequent total repair. Patients were divided into 2 groups depending on the surgical strategy used to choose the surgical approach. In Group I (2007-09), the surgeon-preferred procedure (71%-one-stage total repair) was used. In Group II (2010-14), a surgical algorithm based on pulmonary arterial tree morphology was applied. RESULTS: Median follow-up was 5.04 years and 98% complete. The overall survival rate was 92.59%. Cumulative operative mortality decreased from 10.7% in Group I to 0% in Group II ( P = 0.036). Successful complete repair was performed in 23 (82%) of 28 patients in Group I (median follow-up, 7.99 years) and in 33 (60%) of 55 patients in Group II (median follow-up, 3.85 years). The difference in total survival was not significant between Groups I and II but was markedly influenced by the presence of the 22q11 deletion. The survival of 23 patients with confirmed 22q11 deletion was 73.91%; the survival of 28 patients with confirmed absence of the 22q11 deletion was 100%; and the survival of 32 patients not tested for the 22q11 deletion was 100% ( P = 0.0001). CONCLUSIONS: Total survival was significantly lower in patients with the 22q11 deletion. Surgical management based on preoperative pulmonary arterial anatomical features improves early surgical results.

Predicting outcome in 259 fetuses with agenesis of ductus venosus - a multicenter experience and systematic review of the literature (.).

OBJECTIVE: To evaluate prenatal predictors of postnatal survival in fetuses with agenesis of ductus venosus (ADV). METHODS: This retrospective study reviewed our experience and the literature between 1991 and 2015. Prenatal findings were evaluated and perinatal morbidity and mortality was documented. RESULTS: A total of 259 cases were included in the present analysis from our centers and 49 published studies (15 patients from our retrospective cohort review and 244 from literature review). The intrahepatic and extrahepatic shunts were present in 32.0% (73/226) and 67.7% (153/226), respectively. Cardiomegaly (n = 64/259, 24.7%), hydrops (n = 31/259, 12.0%) and amniotic fluid abnormalities (n = 22/259, 8.5%) were among the most frequent initial ultrasound findings. One hundred and forty-seven fetuses (56.8%) had ADV without structural anomalies while 112 (43.2%) had associated anomalies (cardiac anomalies (n = 66), extra-cardiac anomalies (n = 19) and both cardiac and extra-cardiac anomalies (n = 27)). The mean gestational age (GA) at ultrasound diagnosis was 22.9 ± 6.9 weeks while the mean GA at delivery was 34 ± 7.5 weeks. The overall neonatal survival was 57.1% (n = 148/259). The following factors were associated with survival: advanced maternal age, earlier GA at diagnosis, prematurity, increased nuchal translucency, pericardial effusion, associated cardiac defects (especially AVSD), chromosomal abnormalities, hydrops, hygroma and limb anomalies. CONCLUSION: Fetal hydrops, the presence of associated congenital anomalies and premature delivery are associated with poor prognosis in fetuses with ADV.

Selective management strategy of interrupted aortic arch mitigates left ventricular outflow tract obstruction risk.

BACKGROUND: Left ventricular outflow tract obstruction (LVOTO) is an important problem after interrupted aortic arch (IAA) repair, especially when early reoperation is required during infancy. Several anatomic factors have been identified that increase LVOTO risk; surgical strategies such as concomitant resection of the conal septum or left ventricular outflow tract (LVOT) bypass (single-stage Yasui operation, or staged Norwood procedure, followed by the Rastelli procedure) have been proposed for such patients. METHODS: From 2002 to 2013, a total of 77 neonates underwent IAA repair. Based on the presence of anatomic substrate for LVOTO, patients underwent standard IAA repair and ventricular septal defect closure (n = 53; 69%), concomitant conal resection (n = 7; 9%), or LVOT bypass (n = 17; 22%, staged in 14). We analyzed anatomic details that influence procedure choice, and explored reoperation risk and survival after repair. RESULTS: Neonates who had conal resection or LVOT bypass had significantly smaller aortic valve and subaortic areas, as well as a trend toward a higher prevalence of type B IAA, aberrant right subclavian artery, and bicuspid aortic valve. Freedom from LVOT reoperation was 96% and 80%, at 1 and 8 years, respectively, and was lower after conal resection (P = .008). Most LVOT reoperations (73%) were for discrete subaortic obstruction. Survival was 86% at 8 years, and was not related to procedure choice, whereas freedom from all-cause reoperation was 65% at 8 years, and was higher after standard repair (P = .029). CONCLUSIONS: Compared with published reports, this selective management strategy, which is customized to the degree of aortic valve and subaortic area narrowing, has mitigated and delayed LVOTO risk. With this tailored approach, most LVOT reoperations occur after infancy and are commonly for discrete subaortic obstruction. The effect of aortic valve and LVOT narrowing on increased LVOTO risk is neutralized with LVOT bypass procedures; however, it continues to be the highest after conal resection, suggesting the superiority of LVOT bypass, compared with enlargement in neonates who are at risk of developing LVOTO.

Efficacy of the 'intrapulmonary-artery septation' surgical approach for Fontan candidates with unilateral pulmonary arterial hypoplasia†.

OBJECTIVES: The bilaterally unbalanced development of pulmonary arteries (PAs), as a result of unilateral pulmonary arterial hypoplasia (PAhypo) makes patients either ineligible for the Fontan operation or candidates for the one-lung Fontan operation. In the present study, we examined the efficacy of intrapulmonary-artery septation (IPAS), a technique we reported in 2007 in which a septation is constructed within the central PA, in patients with unilateral PAhypo. METHODS: Sixteen patients with unilateral PAhypo and an affected PA index of ≤60 mm(2)/m(2), including non-confluent PA (NCPA), underwent IPAS between January 2000 and March 2012; patients with pulmonary venous obstruction were excluded from this study. We compared the affected PA index values before and after IPAS and after the Fontan operation as well as the bilateral pulmonary blood flow ratio using pulmonary scintigraphy. The post-Fontan operation values of central venous pressure (CVP), pulmonary vascular resistance (PVR), ventricular end-diastolic pressure (VEDP), cardiac index (CI) and arterial oxygen saturation (SaO2) at the most recent cardiac catheterization, as well as the pre-IPAS and post-Fontan New York Heart Association (NYHA) classification levels were examined. RESULTS: One patient died after IPAS (survival rate, 93.8%). Thirteen (86.7%) of the surviving 15 patients underwent the Fontan operation. The mean PA indexes were 25.5 ± 18.9 mm(2)/m(2) before IPAS and 67.9 ± 34.2 mm(2)/m(2) after IPAS (P = 0.003); the mean PA index was 71.1 ± 50.0 mm(2)/m(2) after the Fontan operation. Restenosis did not occur after the Fontan operation, and the PA diameters were maintained. The mean affected/unaffected lung blood flow ratio was 0.89 ± 0.67. The most recent cardiac catheterization after the Fontan operation indicated the following values: CVP, 11.7 ± 1.8 mmHg; PVR, 1.3 ± 0.4 U m(2); EDP, 5.7 ± 2.0 mmHg; CI, 3.1 ± 0.5 l/min/m(2) and SaO2, 94.9 ± 2.0%. The mean pre-IPAS and post-Fontan NYHA levels were 2.3 ± 0.6 and 1.2 ± 0.4, respectively (P = 0.0002). CONCLUSIONS: With IPAS, the affected PA diameters increased significantly, and were maintained after the Fontan operation, and continuity of the native PAs was achieved. IPAS is very effective for patients suffering from otherwise intractable diseases.

Surgical treatment of varicose veins and venous malformations in Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a mixed mesenchymal malformation characterised by varicose veins, venous and capillary malformations, and hypertrophy of soft tissue and bone. The purpose of this study was to evaluate the surgical outcomes in KTS patients to provide standards for comparison with endovenous therapy. METHODS: The clinical data of consecutive patient with KTS who underwent open venous surgical treatment between January 1987 and December 2008 were reviewed. Demographics, clinical presentation, operative data, and clinical outcomes were recorded. Follow-up information was obtained from the medical records, mailed questionnaires and phone calls. Descriptive statistics, the Kaplan-Meier method and Log-rank statistics were used where appropriate. RESULTS: Twenty-seven females and 22 males, (mean age 26.5 years, range 7.7-55.8) were included in this study. All had varicose veins, 36 (73%) had limb hypertrophy, and 33 (67%) had capillary malformations, with two of three clinical features present in all. The most frequent symptom was pain (N = 43, 88%). Forty-nine patients underwent operations on 53 limbs. Stripping of the GSV, small and accessory saphenous and lateral embryonic veins was performed in 17 (32%), 10 (19%), 9 (17%), and 15 (28%) limbs, respectively. Two patients developed deep vein thrombosis, one had pulmonary embolism (PE), and one patient had peroneal nerve palsy. Freedom from disabling pain at 1, 3 and 5 years was 95%, 77% and 59%, respectively, and freedom from secondary procedures was 78% at 3 years, and 74% at 5 years. At the last follow-up visit, the venous clinical severity score had decreased from 9.48 ± 3.27 to 6.07 ± 3.20 (P < 0.001). CONCLUSIONS: In selected symptomatic patients with KT syndrome, open surgical treatment is safe and durable. Three-fourths of the patients remain free of disabling pain at five years, but secondary procedures are required in one-fourth of the patients. These data can serve as standards for comparison of endovenous therapy for KT syndrome.

Mixed type total anomalous pulmonary venous connection: early results and surgical techniques.

INTRODUCTION: The mixed total anomalous pulmonary connection is a rare type of congenital cardiopathy. The occurrence of multiple connections of the pulmonary veins has important implication in the diagnosis and surgical planning. These types of total anomalous pulmonary venous connection (TAPVC) and the infracardiac have higher surgical mortality. METHODS: Between December 1994 and July 2013, a total of 58 children underwent surgical treatment of TAPVC. Twenty-five (43.1%) patients had supracardiac connection, 15 (25.8%) patients had intracardiac type, 12 (20.6%) patients had a mixed type, and 6 (10.3%) patients had an infracardiac type. In children with mixed TAPVC, four (33.3%) patients had type I, five (41.6%) patients had type II, and three (25.0%) patients had type III. The diagnosis of TAPVC was performed in all patients using Doppler echocardiography, and they underwent cardiopulmonary bypass with aorta-bicaval cannulation and lately we use mild hypothermia with normal flow. The drainage channels were ligated in all patients. RESULTS: The immediate surgical results were as follows: one (16.6%) death in the infracardiac group, four (16.0%) deaths in the supracardiac group, one (8.3%) death in the mixed group, and no death in the cardiac group (no statistical difference, P = .488). CONCLUSION: In this series of patients, the mixed TAPVC could be corrected only with preoperative two-dimensional Doppler echocardiography with good immediate results, and we believe that the use of computed angiotomography combined with three-dimensional reconstruction may improve the surgical outcome mainly in the mixed and infracardiac group of TAPVC.

Multidisciplinary vascular malformations clinic in Hong Kong.

OBJECTIVE: To review clinical characteristics, imaging modalities, and treatment outcomes of patients referred to a multidisciplinary clinic for management of vascular malformations. DESIGN: Retrospective case series. SETTING: Multidisciplinary vascular malformation out-patient referral clinic in a teaching hospital in Hong Kong. PATIENTS: The 141 attendees of the clinic from August 2005 to November 2011. MAIN OUTCOME MEASURES: Management and treatments offered, and responses to treatment. RESULTS: Of the 141 patients, 46% were diagnosed to have low-flow vascular malformations, 16% were diagnosed to have high-flow vascular malformations, and 15% were diagnosed to have a haemangioma. Prior to attending the clinic, approximately one third (32%) of the patients had a clinical diagnosis that was consistent with the final diagnosis. Overall, the radiological and clinical diagnoses were consistent in 43% of the patients. Magnetic resonance imaging and ultrasonography were the most commonly used imaging modalities. Of the 73 patients who received active treatment, 70% had a good response, 12% had minimal improvement, 8% had no change, and 7% had a recurrence or a major complication; in 3% of the patients the outcome was unknown. CONCLUSION: From this retrospective case series, it is evident that confusion still exists over vascular malformations and haemangiomas. Multidisciplinary clinics have a role in providing an accurate diagnosis and facilitating appropriate management and treatment plans. Magnetic resonance imaging and ultrasonography had demonstrable utility in determining the extent of the lesions and flow type.

Outcomes of interrupted aortic arch repair using the carotid artery turndown procedure.

OBJECTIVE: Interrupted aortic arch is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Multiple surgical modalities exist to address this defect. We evaluate the long-term outcome of interrupted aortic arch with the left carotid artery turndown technique from a single institution. METHODS: Patients with interrupted aortic arch who underwent the carotid turndown procedure were identified between September 1982 and March 2010. Medical and surgical records were reviewed. Mortality data were obtained from state death records. RESULTS: Forty-seven patients met inclusion criteria. Median follow-up was 6.2 years (5 days to 23.2 years). Twenty-one patients (45%) had genetic syndromes. There were 4 operative deaths (8.5%) and 9 late deaths (19%). One-year and 5-year survivals were 80.2% and 72.6%, respectively. Seventeen patients (36.2%) required reoperation or other interventions on the aortic arch. CONCLUSIONS: Left carotid artery turndown offers a favorable surgical outcome. It compares with end-to-end repair, while providing a tension-free anastomosis and avoiding neonatal circulatory arrest and cardiopulmonary bypass. Disadvantages include a 2-stage repair and a significant reintervention rate, particularly when compared with the aortic arch advancement technique. Nevertheless, the reduced exposure to circulatory arrest and bypass and avoidance of left bronchial obstruction are important considerations that may offset these limitations.

Clinical characteristics and prognostic factors of primary pulmonary vein stenosis or atresia in children.

BACKGROUND: Primary pulmonary vein stenosis or atresia (PVS/A) is a rare entity with a high mortality rate. The aim of this study was to elucidate the clinical characteristics, progression, and prognostic factors of primary PVS/A in children. METHODS: We reviewed the medical records of patients who had primary PVS/A with normally connected pulmonary veins (PVs) at five pediatric cardiology centers in Korea between 1995 and 2010. RESULTS: A total of 34 cases were identified. The median age at diagnosis was 12.0 months. During the follow-up period (median, 18 months; range, 2 to 185 months), PVS/A progressed to previously uninvolved veins in 9 patients. Surgical interventions were performed on 29 patients (venoplasty on 25 and pneumonectomy on 4). Nineteen of the patients who underwent venoplasty had restenosis after a median of 2 months. The sutureless technique did not reduce the rate of restenosis, progression of the disease to previously uninvolved PVs, or mortality rate. The mortality rate was 46.7%, the median age of death was 10.8 months, and the median interval between diagnosis and death was 3.0 months. In univariate analysis, predictors of death included involvement of at least three PVs, bilateral PV involvement, infancy-onset PVS/A, restenosis after surgery, and progression to previously uninvolved PVs. In multivariate analysis, significant risk factors for death were involvement of at least three PVs (hazard ratio, 8.8; p < 0.0001) and progression to uninvolved PVs (hazard ratio, 4.2; p = 0.014). CONCLUSIONS: Primary PVS/A may carry a significant risk of recurrent and progressive PV obstruction or death even after surgical venoplasty.