Surgical management of pulmonary artery sling in children Yong, Matthew S., BMedSci; d'Udekem, Yves, MD, PhD; Brizard, Christian P., MD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
04/2013, Letnik:
145, Številka:
4
Journal Article
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Objectives Pulmonary artery (PA) sling is a rare vascular anomaly associated with congenital tracheal stenosis. The natural history is poor and these patients often require early surgical ...intervention. We describe our experience with repair of this condition. Methods From 1984 to 2011, 21 patients with PA sling underwent repair at the Royal Children's Hospital (median age, 5.9 months). PA sling was associated with compression of the trachea in all patients. Tracheal surgery was required in 12 (57.1%) patients. All patients had an echocardiogram, and concomitant repair of coexisting cardiac anomalies was performed in 6 (28.6%, 6/21) patients. Results Operative mortality was 14.3% (3/21), occurring at 19 days, 4.4 months, and 5 months after surgery. Operative mortality for the first 10 years was 22.2% (1984-1993; 2/9), the next 10 years was 14.3% (1994-2003; 1/7), and 0% for the most recent 7 years (2004-2011; 0/5). All deaths occurred in patients requiring tracheal repair (25%, 3/12). No deaths have occurred since 2004 with introduction of the slide tracheoplasty technique. One (5.6%, 1/18) late death occurred at 8 months after repair. After tracheal repair, intervention for excessive granulations and tracheomalacia was necessary in 6 (50%, 6/12) patients. Median follow-up was 8 years (mean, 8.6 ± 6.4 years; range, 5 months to 20.6 years), and all survivors (100%, 17/17) remain asymptomatic. Conclusions Children with PA sling who do not require tracheal surgery have excellent outcomes. Mortality is determined by the need for tracheal surgery. However, with the advent of the slide tracheoplasty technique, mortality can be reduced. Survival beyond 1 year after surgery offers excellent prognosis.
The extent to which topically applied solid nanoparticles can penetrate the stratum corneum and access the underlying viable epidermis and the rest of the body is a great potential safety concern. ...Therefore, human epidermal penetration of a novel, transparent, nanoparticulate zinc oxide sunscreen formulation was determined using Franz-type diffusion cells, 24-hour exposure and an electron microscopy to verify the location of nanoparticles in exposed membranes. Less than 0.03% of the applied zinc content penetrated the epidermis (not significantly more than the zinc detected in receptor phase following application of a placebo formulation). No particles could be detected in the lower stratum corneum or viable epidermis by electron microscopy, suggesting that minimal nanoparticle penetration occurs through the human epidermis.
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with limited data on long-term outcomes after surgical intervention.
We conducted a retrospective ...review of all children (N = 42) who underwent surgical repair of ALCAPA between 1980 and 2014 at the Royal Children's Hospital, Melbourne.
Twenty-nine (69% 29 of 42) patients underwent coronary reimplantation, 12 (29% 12 of 42) had intrapulmonary baffle (Takeuchi) repair, and 1 (2% 1 of 42) patient had ligation of the anomalous coronary artery. Nine (21%, 9 of 42) patients had concomitant mitral valve (MV) repair at the time of ALCAPA repair. A left ventricular assist device (LVAD) was used in 36% (15 of 42) of patients. Early mortality was 2.4% (1 of 42 patients). Median follow-up was 14 years (mean, 13 years; range, 4 months-31 years). There were no late deaths. Survival was 98% at 20 years. Freedom from reoperation was 81%, 81%, and 76% at 5, 10, and 20 years after operation, respectively. Eight patients underwent late MV repair or replacement at a median of 3 years (mean, 8 years; range, 2 months-25 years) after operation. Freedom from late MV repair or replacement was 86% at 5 and 10 years and 81% at 20 years after operation. Eleven (26% 11 of 42) patients had severe mitral regurgitation (MR) preoperatively. Of those 11 patients, 5 (45% 5 of 11) had concomitant MV repair at the time of ALCAPA repair, 3 (27% 3 of 11) had late MV repair or replacement, and the remaining 3 (27% 3 of 11) patients had mild MR at last follow-up. Thirty-six (90% 36 of 41) patients had normal left ventricular function and 4 (10% 4 of 41) patients had mildly reduced left ventricular (LV) function at last follow-up.
ALCAPA can be operated on with good outcomes. Persistent MR and a moderate rate of late MV repair warrants close follow-up.
Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge as it is often associated with severe obstruction. We describe a large cohort of neonates who underwent ...TAPVD repair at a single institution.
From 1973 to 2008, 112 patients underwent simple TAPVD surgery during the first month of life. Data collection occurred retrospectively.
Preoperative pulmonary venous obstruction (PVO) occurred in 89 (79.5%) patients. There were 12 (10.7%) early deaths. Significant risk factors were bypass time greater than 65 minutes (p=0.014) and emergent surgery (p=0.005). Hospital mortality was unchanged throughout the 3 eras (1973 to 1988, 1989 to 1998, 1999 to 2008), despite an increase in patients with preoperative acidosis (p=0.004) and severe TAPVD obstruction (p=0.038) during the recent 10 years. There were 6 (6.25%) late deaths within 2 years of repair. Survival at 20 years was 83.4% (95% confidence interval 75 to 89). Risk factors for late death were operative weight 2.5 kg or less (p=0.004) and postoperative pulmonary hypertensive crisis (p=0.02). Reoperation for recurrent PVO was required in 13 patients (11.9%). Risk factors were operative weight 2.5 kg or less (p=0.035) and postoperative pulmonary hypertensive crisis (p=0.002). Follow-up was 96% complete and survivors (n=90) were asymptomatic at a median age of 11.7 years.
Hospital mortality remained unchanged over the 36-year period. Survival beyond 2 years offers excellent outcome. Risk factors for mortality were the preoperative clinical status, prolonged bypass time, persisting micro-obstruction, and low operative weight. A reduction in mortality will likely require development of effective medical management for patients who have peripheral PVO not amenable to surgical repair.
Background The long-term impact of fenestration at the time of Fontan operation remains unclear. We aimed to review the early and long-term impact of Fontan fenestration in the Australia and New ...Zealand cohort. Methods and Results We reviewed 1443 patients (621 fenestrated, 822 nonfenestrated) from the Australia and New Zealand Fontan registry. Data were collected on preoperative demographics, operative details, and follow-up. Propensity-score matching was performed to account for the various preoperative and operative differences and risk factors. Primary outcomes were survival and freedom from failure. Median follow-up was 10.6 years. After propensity-score matching (407 matched pairs), there was no difference in survival (87% versus 90% at 20 years;
=0.16) or freedom from failure (73% versus 80% at 20 years;
=0.10) between patients with and without fenestration, respectively. Although patients with fenestration had longer bypass and cross-clamp times (
<0.001), there was no difference in hospital length of stay or prolonged pleural effusions (
=0.80 and
=0.46, respectively). Freedom from systemic and Fontan circuit thromboembolism was higher in the nonfenestrated group (89%; 95% CI, 88%-95%) than the fenestrated group (84%; 95% CI, 77%-89%;
=0.03). There was no difference in incidence of plastic bronchitis, protein-losing enteropathy, New York Heart Association Class III/IV symptoms, or Fontan takedown. Conclusions In the propensity score-matched analysis we have demonstrated no difference in long-term survival or freedom from Fontan failure in patients with and without fenestration. There was a higher incidence of long-term thromboembolic events in patients with fenestration. Overall, it appears that fenestration in Fontan circulation does not bring long-term benefits.
Intramural coronary arteries may complicate coronary artery transfer during the arterial switch operation. We sought to determine the long-term outcomes of 28 patients with intramural coronary ...arteries who underwent an arterial switch operation at a single institution.
All patients who had intramural coronary arteries and underwent an arterial switch operation were identified from the hospital database and retrospectively reviewed.
From 1983 to 2009, 720 patients underwent an arterial switch operation at our institution. Twenty-eight (3.9%, 28 of 720) had intramural coronary arteries. Patients with intramural coronary arteries had transposition of the great arteries (96%, n = 27) or Taussig-Bing anomaly (4%, n = 1). There were no deaths. Follow-up was 100% complete. Mean follow-up was 16.3 years (median, 15.5 years; range, 5.6 to 26.9 years). No patient required reoperation or catheter reintervention on the coronary arteries. Freedom from reoperation was 93% at 10 years. No patient had more than mild aortic regurgitation at last follow-up. Nine (32%, 9 of 28) patients had coronary angiograms at median 16 months (range, 14 months to 17 years) after arterial switch operation. All patients were asymptomatic at the time of angiogram. One patient had mild stenosis of the circumflex coronary artery demonstrated on a routine coronary angiogram 14 months postoperatively. All 28 patients were asymptomatic and in New York Heart Association functional class I at last follow-up.
Patients with intramural coronary arteries are not at increased risk of death or coronary reinterventions and have excellent late outcomes after the arterial switch operation.
Absent pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and may lead to significant respiratory compromise. We ...describe the outcomes of surgical correction of absent pulmonary valve syndrome and risk factors for mortality and reoperation.
A review of 52 patients with absent pulmonary valve syndrome who underwent surgical correction between 1975 and 2013 was conducted. The median age and weight at repair were 9 months (range, 4 days to 24.2 years) and 6.9 kg (range, 1.8 to 56 kg). Preoperative intubation was required in 15 patients (29%), and 21 patients (40%) underwent urgent repair. The pulmonary valve was replaced with a valved conduit in 16 patients (31%) or monocusp valve in 16 patients (31%). Valveless repair was performed in 20 patients (38%). Pulmonary artery reduction was performed in 39 patients (75%), and 2 patients (4%) underwent a Lecompte maneuver.
The median follow-up time was 13 years (range, 1 month to 35 years). Early mortality was 18.8% (3 of 16) during 1975 through 1989, 19% (4 of 21) during 1990 through 2000, and 0% (0 of 15) during 2001 through 2013. Late mortality was 6.7% (3 of 45). Overall survival at 5, 10, and 20 years was 81.4%±5.6%. On multivariate analysis, preoperative ventilation (p=0.009) was the only risk factor for overall mortality. Freedom from late reoperation at 5, 10, and 20 years was 79.7%±6.9%, 69.4%±8.2%, and 52.1%±9.8%, respectively. No difference in reoperation rates was found between valved conduit, monocusp, or valveless techniques. Risk factors for late reoperation on multivariate analysis were prematurity (p=0.001) and neonatal primary repair (p=0.007). Longer postoperative ventilation periods were predicted by preoperative ventilation (p<0.001) and surgery during infancy (p=0.01).
Long-term survival for absent pulmonary valve syndrome has improved during the last decade. Preoperative ventilation predicted longer postoperative ventilation and mortality.
This study analyzed a protocol of neonatal rehabilitation of hypoplastic pulmonary arteries in the management of pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary ...collateral arteries (MAPCAs). Ideal management of patients with pulmonary atresia, VSD, and MAPCAs is the subject of controversy.
From June 2003 to December 2008, 25 consecutive patients were diagnosed with pulmonary atresia, VSD, and MAPCAs, and 20 were entered into a neonatal shunting regimen. The median age at the first operation was 3.6 weeks (range, 0.7 to 17 weeks). All patients underwent an initial central or modified Blalock-Taussig shunt, or both. Further preparatory procedures included 26 pulmonary artery patch reconstructions, 19 right ventricle-to-pulmonary artery conduits, 4 MAPCA ligations, and 4 further shunts. No patient underwent translocation of the collateral arteries.
At the latest follow-up, 12 of 20 patients have had a complete repair at a median age of 18 months (range, 11 to 48 months), 6 are awaiting repair, and 2 are considered unlikely to be repaired. No patient was missing follow-up. Median pulmonary artery indices had grown from 14.51 to 118.7 in 17 patients. Postoperative angiograms were performed at a median of 8 months (range, 1.9 to 32.7 months) in 9 of 12 completely repaired patients. The median right/left ventricular pressure ratio was 0.64 (range, 0.54 to 0.91).
Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival.
Aortic dissection and rupture in adolescents after tetralogy of Fallot repair Konstantinov, Igor E., MD, PhD; Fricke, Tyson A., BMedSci; d'Udekem, Yves, MD, PhD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
11/2010, Letnik:
140, Številka:
5
Journal Article
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