Viktor Hraska

Use of rapid-deployment extracorporeal membrane oxygenation for the resuscitation of pediatric patients with heart disease after cardiac arrest.

INTRODUCTION We have recently used extracorporeal membrane oxygenation as a means of rapidly resuscitating pediatric patients with heart disease after cardiopulmonary arrest, in whom conventional resuscitation measures have failed. METHODS We developed a fully portable extracorporeal membrane oxygenation circuit that is maintained vacuum and carbon dioxide-primed at all times. When needed, the circuit is crystalloid-primed and can be ready for use within 15 minutes. Since February 1996, we have used this rapid-deployment circuit to resuscitate 11 pediatric patients in full cardiopulmonary arrest. RESULTS The median age of the 11 patients was 120 days (2 days to 4.6 years). Nine patients had a cardiac arrest after cardiac surgery. One patient had a cardiac arrest during cardiac catheterization and one patient had a cardiac arrest before cardiac surgery. Median duration of cardiopulmonary resuscitation was 55 minutes (range 20 to 103 minutes), with no difference in the duration of cardiopulmonary resuscitation between survivors and nonsurvivors. Ten of 11 patients (91%) were weaned from extracorporeal membrane oxygenation and seven (64%) survived to hospital discharge. Six patients are long-term survivors, five of whom are in New York Heart Association class I; one survivor is in class II. Seven patients resuscitated with extracorporeal membrane oxygenation before the use of this rapid-deployment circuit had a median duration of cardiopulmonary resuscitation of 90 minutes, with two (28.6%) survivors. CONCLUSIONS The use of rapid-deployment extracorporeal membrane oxygenation results in shorter resuscitation times and improved survival in pediatric patients with heart disease after cardiopulmonary arrest.

Scimitar Syndrome A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study

Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used. # Clinical Perspective {#article-title-44}Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

Surgery for complications of trans-catheter closure of atrial septal defects: a multi-institutional study from the European Congenital Heart Surgeons Association

OBJECTIVE This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). METHODS The records of all (n=56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. RESULTS A wide range of ASD sizes (5-34mm) and devices of various types and sizes (range 12-60mm) were involved, including 13 devices less than 20mm. Complications leading to surgery included embolisation (n=29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n=12), significant residual shunt (n=12), aortic or atrial perforation or erosion (n=9), haemopericardium with tamponade (n=5), aortic or mitral valve injury (n=2) and endocarditis (n=1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% (p=0.001). CONCLUSIONS Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory.

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study

Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate ( P= 0.006). Overall mortality was associated to malignancy ( P =0.0008), and adverse events during follow-up ( P =0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas. # Clinical Perspective {#article-title-51}Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ⩽18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

Intermediate results with correction of tetralogy of Fallot with absent pulmonary valve using a new approach

OBJECTIVE The intermediate results achieved with a new technique for primary repair of tetralogy of Fallot with absent pulmonary valve syndrome are reported. METHOD Apart from correction of tetralogy of Fallot, this approach includes translocation of the pulmonary artery anterior to the aorta and away from the tracheobronchial tree. Since November 1998 this technique has been employed in three symptomatic newborns and three infants with a diagnosis of tetralogy of Fallot with absent pulmonary valve. The median age at surgery was 37 days (range 14-256 days). The median weight at operation was 3.4 kg (range 2.9-4 kg). All patients had severe respiratory problems and congestive heart failure with cyanosis. RESULTS There was no early or late death during the follow-up (median 27 months). One patient required redo due to failure to thrive as a consequence of right ventricle volume overload. Valved conduit was inserted to pulmonary position. Respiratory symptoms disappeared or were significantly reduced in all patients. Postoperative computed tomographic scan showed no compression of trachea and main bronchi, pulmonary artery was away from tracheobronchial tree in all patients. Patients are doing well with adequate growth. CONCLUSIONS The new technique described here has a potential to reduce or eliminate bronchial compression by pulmonary artery. Translocation of pulmonary artery anterior to the aorta takes the dilated pulmonary artery away from the trachea and bronchial tree. This approach was found to be technically feasible and can be useful especially in symptomatic newborns and infants.

A new approach to correction of tetralogy of Fallot with absent pulmonary valve

A new technique for primary repair of tetralogy of Fallot with absent pulmonary valve syndrome is suggested. In addition to the standard intracardiac portion of tetralogy of Fallot repair, this approach includes translocation of the pulmonary artery anterior to the aorta, shortening of the ascending aorta, and shortening and plication of the pulmonary artery. This new technique should eliminate compression of the tracheobronchial tree caused by impingement by the pulmonary artery.

The Long-Term Outcome of Open Valvotomy for Critical Aortic Stenosis in Neonates

BACKGROUND This study analyzed the long-term outcome after operations for open aortic valvotomy, specifically looking at the preservation of the native aortic valve. METHODS Between 1983 and 2011, 34 neonates with critical aortic stenosis underwent open valvotomy. The mean age at operation was 15 days (range, 1 to 28 days). Follow-up was 100% complete. RESULTS Three patients died early. The probability of survival was 91.2%, with a mean follow-up of 11 years (range, 10 months to 21 years). Event-free survival was 77%, 68%, 68%, and 57% at 5, 10, 15, and 20 years, respectively, and freedom from aortic valve replacement was 93%, 83%, 68%, and 57%. Tricuspid valve morphology revealed the best outcome, with event-free survival of 90% (p = 0.006) and 100% freedom from aortic valve replacement (p = 0.0012) at 20 years of follow-up. All but 4 patients were in New York Heart Association class I; 77 % of all patients were without medication. CONCLUSIONS In neonates, predictable and consistent long-term results can be accomplished by open valvotomy in any type of valve morphology, even in an asymmetric arrangement. Operative repair not only offers a 90% survival benefit in the long-term but also preserves the native aortic valve in most patients. Clearly superior results were achieved in tricuspid valve morphology, in which there was no need for aortic valve replacement at 20 years of follow-up.

Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature.

Best evidence protocol was applied and the question addressed, whether there is an outcome advantage of a hybrid approach including bilateral pulmonary banding (BPB) and ductal stenting over Norwood procedure (NP) to maintain systemic circulation and to restrict pulmonary blood flow in neonates with hypoplastic left heart syndrome (HLHS). Out of 80 articles published since the original description of BPB in 2002, eight were selected to answer the clinical question. All studies were retrospective case-series after BLB, only three compared results with NP controls, but not in a randomized fashion. Only three chose high-risk patients. Mean follow-up interval was mentioned only in one study. Reported mean hospital, interstage, and stage 2 mortalities after BPB was 17.3, 12.4, and 25.3%, respectively. Estimated transplant-free survival beyond stage 2 palliation ranged from 7 to 80%, with no significant differences to NP controls in three studies. Reintervention rate was high, ranging from 20 to 42% at different sites. Due to study design or inhomogeneity of patient groups, current literature does not show evidence that bilateral pulmonary banding improves outcome with respect to survival beyond second-stage HLHS palliation.

Survival and reintervention after neonatal repair of truncus arteriosus with valved conduit

OBJECTIVE Neonatal primary repair has progressively become the treatment of choice for truncus arteriosus with encouraging survival. However, use of valved conduits to reconstruct the right ventricular outflow tract (RVOT) inevitably induces reintervention. This study estimates survival and rate of catheter-interventional and surgical reinterventions. METHODS Thirty-five consecutive neonates who underwent truncus repair with 27 homografts and 8 Contegras from 1987 to 2007 were studied. Interrupted aortic arch (IAA) was associated in nine patients. Actuarial survival and freedom from reintervention were evaluated according to Kaplan-Meier method. RESULTS Five patients died early after repair. Two died late and one death was related to reintervention. Survival was 91.9%+/-5.4% from postoperative month 2 onwards when IAA was not associated and 41.7%+/-17.3% from month 4 in IAA presence. During a median follow-up of 68 months (range 1-180 months), 42 reinterventions (of which 17 reoperations) were performed in 21 patients. Rate of reintervention was 2.6 per early survivor per 10 years. RVOT obstruction constituted the main indication: branch pulmonary arteries often being involved (n=25). Uncommon indication was subaortic stenosis (n=3), aortic arch obstruction (n=2) and truncal valve regurgitation (n=2). At year 10, freedom from first, second and third reintervention was 17.9 %+/-8.1%, 46.1%+/-10.6% and 81.9%+/-9.5%, respectively. Sixteen first conduits were explanted. Freedom from first conduit replacement was 87.5%+/-6.8%, 64.1%+/-10.2% and 39.5%+/-10.7% at year 1, 3 and 5, respectively. Homografts enjoyed higher durability than Contegras. CONCLUSION Neonatal repair of truncus arteriosus results in high survival, the only risk being IAA association. The rate of reintervention is heavily influenced by stenosis of branch pulmonary arteries.

Does the shunt type determine mid-term outcome after Norwood operation?

OBJECTIVES With improved short-term outcomes the right ventricular to pulmonary artery shunt (Sano) has become the preferred pulmonary blood source in the Norwood procedure in many centres. However, most studies analysed consecutive cohorts, with a first modified Blalock-Taussig shunt (BT) followed by the Sano cohort. Besides, neither comprehensive preoperative risk analysis nor outcome beyond 1 year of age was investigated. METHODS This study reviews 109 neonates undergoing the Norwood procedure in the same interval between October 2002 and December 2009. The Sano (38) or BT shunt (71) was assigned according to the surgeons preference. Two neonates subsequently underwent successful biventricular repair and were excluded. The Aristotle comprehensive score (ACS) was used to evaluate preoperative risk, with high-risk patients (n = 39) classified as having an ACS ≥ 20, and low-risk patients (n = 68) given an ACS <20. RESULTS Mean Aristotle score at the Norwood operation was 18.8 ± 0.4 and 18.9 ± 0.3 (P = 0.9) in Sano and BT, respectively. Mean follow-up interval was 4.1 ± 2.1 years (range: 1.7-8.9 years). Actuarial survival was similar, stabilizing from the 8th postoperative month onwards at 78.6 ± 4.9% (95% CI: 67.0-86.5%) for Sano and 78.4 ± 6.8% (95% CI: 61.4-88.6%) for BT; P = 0.95. Midterm actuarial survival was higher in low-risk patients, 88.2 ± 3.9% (95% CI: 77.8-93.9%) than in high-risk patients: 61.5 ± 7.8% (95% CI: 44.5-74.7%, P = 0.0003). No survival benefit was detected in low- or high-risk cases for either shunt type. Risk factors for midterm mortality were cardiorespiratory failure requiring ventilation (13/34, P = 0.004), and ACS ≥ 20 (15/39, P = 0.001), but not shunt type (8/37, P = 0.95). Increased number of shunt-related interventions before the Glenn procedure were noted with Sano (32.4 versus 6.5%, P = 0.002). CONCLUSIONS Preoperative risk factors, regardless of shunt type, influence midterm survival after the Norwood procedure with an excellent outcome in low-risk patients, while high-risk cases still incur a significant mortality. Sano shunt interventions occurred with increased numbers. Although, Sano shunt may be the only feasible option in some instances, given the possible negative effects of ventriculotomy on right ventricle function, the widespread use of Sano shunt should be reconsidered.