Superior results following the Ross procedure in patients with congenital heart disease.

BACKGROUND AND AIM OF THE STUDY: The Ross procedure is a versatile operation that can be applied for aortic valve replacement (AVR) in patients with congenital heart disease (CHD), including small infants and those with complex left ventricular outflow tract (LVOT) obstruction. Herein, the clinical outcome is reported following the Ross procedure in patients with CHD at the authors' institution. METHODS: The medical records of patients who underwent the Ross procedure for CHD between 1991 and 2007 were reviewed. A competing-risks methodology was used to determine the time-related prevalence and associated factors for three mutually exclusive end states after the Ross procedure, namely death prior to subsequent cardiac reoperation, cardiac reoperation, and survival without subsequent reoperation. RESULTS: A total of 151 patients (98 males, 53 females) was identified. The median age at the time of surgery was 8.6 years (range: 4 days to 33 years). Previously, 103 patients (68%) had undergone cardiac interventions, and 43 (28%) required LVOT enlargement (modified Ross-Konno procedure). A competing-risk analysis showed that, at 10 years after the Ross procedure, 8% of patients had died without subsequent reoperation, 26% underwent cardiac reoperation, and 66% remained alive without further reoperation. The 10-year freedom from autograft and homograft reoperation was 95% and 71%, respectively. Factors associated with early risk of mortality were age < 1 year and no prior surgical/percutaneous intervention at the time of the Ross procedure. Surgical factors associated with cardiac reoperation were concurrent cardiac surgery and the use of fresh homografts. There were no bleeding or thromboembolic complications, and the 15-year freedom from endocarditis was 95%. Ultimately, 99% of the survivors were in NYHA class I or II. CONCLUSION: The Ross procedure remains the authors' procedure of choice for AVR in patients with CHD. Outcomes in infants aged < 1 year may improve with better patient selection and palliative surgical/percutaneous interventions prior to valve replacement. The late survival was excellent and valve-related complications were minimal. The high autograft longevity led to few patients requiring late reoperation for graft replacement.

Treatment of status epilepticus in children.

Status epilepticus is a medical emergency that requires prompt recognition and urgent treatment. Successful management of status epilepticus in children depends upon implementation of a treatment protocol modified according to current practices as well as availability of antiepileptic medications, out of hospital emergency services and intensive-care facilities. We propose a treatment protocol for status epilepticus in children in the Kingdom of Saudi Arabia.

Outcomes of Damus-Kaye-Stansel anastomosis at time of cavopulmonary connection in single ventricle patients at risk of developing systemic ventricular outflow tract obstruction.

OBJECTIVES: Children with various single ventricle anomalies are at risk of developing systemic ventricular outflow tract obstruction (SVOTO) following volume unloading with cavopulmonary connection (CPC). We aim to evaluate the value of Damus-Kaye-Stansel (DKS) anastomosis at the time of CPC in eliminating late SVOTO risk. METHODS: Retrospective review of single ventricle patients who underwent DKS concurrent with CPC between 1997 and 2012 was performed. Clinical, echocardiographic and angiographic outcomes were analysed. RESULTS: Thirty-six children with single ventricle underwent DKS at the time of Glenn bidirectional CPC (n = 29) or Fontan total CPC (n = 7). The underlying anatomy was double inlet left ventricle (n = 18), double outlet right ventricle (n = 8), unbalanced atrioventricular septal defect (n = 4) and other (n = 6). Prior palliation included pulmonary artery band (n = 35), coarctation/arch repair (n = 11) and atrial septectomy (n = 8). Median age at the time of DKS was 8.9 months (range 3.6 months-9.1 years) and the median weight was 6.7 kg (range 5-27 kg). At the time of DKS, 17 patients (47%) had no SVOT gradient and 19 (53%) had SVOT gradient (mean 23.4 ± 18.7 mmHg). Overall survival was 89 and 83% at 1 month and 5 years, respectively. None of the deaths were related to SVOTO or DKS complications. When present, SVOT gradient decreased from 23.4 ± 18.7 mmHg preoperatively to 0 after DKS (P < 0.001). At the last follow-up, none of the patients developed any SVOT gradient; 78% of them had zero or trivial aortic/neoaortic valve regurgitation while 22% had mild regurgitation. None of the patients had evidence of compression of the left pulmonary artery or bronchus. Eighty-one percent of patients have reached or are suitable candidates awaiting final palliative surgery. CONCLUSIONS: DKS can be safely performed in conjunction with CPC without added mortality risk. It is very effective in mitigating SVOTO risk, with sustainable good semilunar valves function. Our data support an aggressive approach to performing DKS concurrent with CPC in children with single ventricle pathologies at risk of developing SVOTO.

Results of rapid-response extracorporeal cardiopulmonary resuscitation in children with refractory cardiac arrest following cardiac surgery.

OBJECTIVES: Survival of children having cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) is very poor. We sought to examine current era outcomes of extracorporeal CPR (ECPR) support for refractory arrest following surgical correction of congenital heart disease. METHODS: Demographic, anatomical, clinical, surgical and support details of children requiring postoperative ECPR (2007-12) were included in multivariable logistic regression models to determine the factors associated with survival. RESULTS: Thirty-nine children, median age 44 days (4 days-10 years), required postoperative ECPR at a median interval of 1 day (up to 15 days) after surgery. Thirteen (33%) children had single-ventricle pathology; Risk Adjustment in Congenital Heart Surgery (RACHS)-1 categories were 2, 3, 4 and 6 in 6, 15, 13 and 5 patients, respectively. Median CPR duration was 34 (8-125) min, while median support duration was 4 (1-17) days. Seven (18%) patients underwent cardiac re-operation, 28 (72%) survived >24 h after support discontinuation and 16 (41%) survived. Survival rates in neonates, infants and older children were 53, 39 and 17% (P=0.13). Survival rates for single- vs two-ventricle pathology patients were 54 and 35%, (P=0.25) and 50, 47, 23 and 60% in RACHS-1 2, 3, 4 and 6 patients, respectively (P=0.37). Survivors had shorter CPR duration (25 vs 34 min, P=0.05), lower pre-arrest lactate (2.6 vs 4.6 mmol/l, P=0.05) and postextracorporeal membrane oxygenation (ECMO) peak lactate (15.4 vs 20.0 mmol/l, P<0.001). On multivariable analysis, factors associated with death were higher immediate post-ECMO lactate (odds ratio, OR 1.34 per mmol/l, P=0.008) and renal failure requiring haemodialysis (OR 14.1, P=0.01). CONCLUSIONS: ECPR plays a valuable role in children having refractory postoperative cardiac arrest. Survival is unrelated to cardiac physiology or surgical complexity. Timely support prior to the emergence of end-organ injury and surgical correction of residual cardiac lesions might enhance survival.

Cardiac reoperations following the Ross procedure in children: spectrum of surgery and reoperation results.

OBJECTIVES: The Ross procedure is the preferred aortic valve replacement (AVR) choice in small children. Nonetheless, it is a complicated surgery and there are concerns that subsequent cardiac reoperations are exceptionally complex and associated with high morbidity and mortality. We examine the surgical spectrum and report outcomes of cardiac reoperations in patients who had undergone the Ross procedure during childhood. METHODS: Records of 227 consecutive children (<18 years old) who had undergone the Ross procedure at our institution from 1991 to 2004 were reviewed. Our patient cohort was 50 patients who underwent 58 cardiac reoperations following the Ross procedure during the follow-up. Time-related outcomes were analyzed. RESULTS: From 1992 to 2009, 50 patients, 37 males (74%), underwent cardiac reoperation at a mean age of 15.6±5.2 years and a mean interval of 3.9±3.0 years following the Ross procedure. Risk factors for cardiac reoperation following the Ross procedure on multivariable analysis were rheumatic fever, aortic regurgitation, concomitant cardiac surgery, use of fresh homografts and earlier era of surgery. Overall, 32 (55%) reoperations were isolated procedures whereas 26 (45%) were more complex involving 2-4 simultaneous cardiac procedures. In total, 92 procedures were performed including AVR (n=31), homograft replacement (n=23), mitral valve replacement (n=18), mitral valve repair (n=11), tricuspid valve repair (n=5) and other (n=4). There was no operative mortality and one late death. Survival was 98% at 10 years. During the follow-up, 8 of 50 patients required further cardiac surgery following initial reoperation with freedom from additional cardiac surgery of 82% at 10 years. Subsequent cardiac surgery risk was higher in patients with pre-operative aortic regurgitation and those who had concomitant surgery at time of Ross on log-rank analysis. Among survivors, 96% are in New York Heart Association class I/II. CONCLUSIONS: A wide range of cardiac reoperations may be required in children following the Ross procedure, especially those with underlying rheumatic aetiology, aortic regurgitation and multivalvular involvement. Despite complexity, reoperation following the Ross procedure can be performed with low mortality and good mid-term results. This information should be taken into consideration during the selection of aortic valve substitute in children.

Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection.

OBJECTIVES: Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. METHODS: Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. RESULTS: Median age was 32 days (range, 15-118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53% had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10% had impaired ventricular function, 11% had moderate atrioventricular valve regurgitation, and 32% had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39% had died, and 57% underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15% had died, and 85% underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53%. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre-bidirectional cavopulmonary connection saturation. CONCLUSIONS: Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

Outcomes and associated risk factors for mitral valve replacement in children.

OBJECTIVE: We aim to report time-related outcomes following mitral valve replacement (MVR) in children and to identify factors affecting outcomes. METHODS: Clinical records from 307 children who underwent MVR between 1985 and 2004 were reviewed. Competing-risks methodology determined time-related prevalence of three mutually exclusive end-states: death, mitral reoperation and survival without subsequent MVR, and their associated risk factors. RESULTS: Mean age was 11.4 ± 5.6 years including 36 (12%) patients < 2 years old. There were 154 (50%) males. Underlying pathology was rheumatic fever (n = 195, 64%), congenital (n = 83, 27%) and other (n = 29, 9%) with congenital pathology predominant in younger children while rheumatic fever predominant in older children. Hemodynamic manifestation was regurgitation (83%), stenosis (5%), or mixed disease (12%). One hundred and twenty-six patients (41%) had undergone a prior cardiac surgery including mitral surgery (n = 96, 31%). Initial mitral prosthesis was mechanical (n = 229, 75%), tissue (n = 71, 23%), or homograft (n = 7, 2%). Concomitant cardiac surgery was required in 141 patients (46%). Competing-risks analysis predicted that 20 years following MVR, approximately 17% of patients have died, 51% have undergone mitral reoperation and only 33% were alive and free from mitral reoperation. Risk factors for death without mitral reoperation included younger age < 3 years [PE (parameter estimates): +1.66 ± 0.31, p < 0.001], longer cross-clamp time (PE: +0.11 ± 0.04/10 min, p = 0.005), postoperative complications (PE: +1.5 8 ± 0.31, p < 0.001), and higher prosthesis size/body surface area (BSA)-predicted mitral annulus ratio (PE: + 0.48 ± 0.10, p < 0.001). Risk factors for mitral reoperation included implantation of homograft or tissue prosthesis (PE: +1.12 ± 0.23, p < 0.001) and smaller prosthesis size (PE: +0.06 ± 0.03/1 mm, p = 0.05). Fifteen-year freedom from pacemaker implantation, endocarditis, bleeding, and thromboembolism was 92%, 96%, 82%, and 92%, respectively. CONCLUSIONS: Mortality and mitral reoperation are common after MVR in children and outcomes can be predicted based on patient's age, prosthesis size, and other associated factors. Some modifiable factors such as avoiding oversized prostheses may improve outcomes especially in the smallest children.

The rastelli procedure for transposition of the great arteries: resection of the infundibular septum diminishes recurrent left ventricular outflow tract obstruction risk.

BACKGROUND: The Rastelli procedure is the standard surgical treatment of d-transposition of great arteries (d-TGA), ventricular septal defect (VSD), and pulmonary stenosis. Late morbidity is significant due to recurrent left ventricular outflow obstruction (LVOTO), early conduit obstruction, and arrhythmias, with troublesome late mortality. To avoid recurrent LVOTO, we routinely enlarge the VSD and resect the infundibular septum before LV baffling to the aorta. We examined the efficacy of this approach in mitigating recurrent LVOTO risk. METHODS: Late echocardiographic and time-related clinical results of patients undergoing the Rastelli procedure were examined. Demographics and operative variables affecting outcomes were analyzed. RESULTS: The Rastelli cohort comprised 36 patients with d-TGA, VSD, and pulmonary stenosis. Median age at operation was 2.4 years (range, 0.3 to 8.3 years). Pulmonary stenosis was present in 31 and atresia in 5. Twenty-two patients had undergone a previous aortopulmonary shunt, and 6 had an atrial septectomy. No operative or late deaths occurred. Time-related freedom from permanent pacemaker implantation, recurrent LVOTO on echocardiogram, and conduit replacement at 10 years was 82%, 100%, and 49%, respectively. Systolic function was normal in all but 3 patients and 92% were in New York Heart Association functional class I and II. None of the patients had late arrhythmias or required heart transplantation. CONCLUSIONS: Early and midterm survival after the Rastelli procedure is satisfactory. Aggressive resection of the infundibular septum to enlarge the VSD has mitigated the risk of LVOTO recurrence. Late conduit obstruction remains an important source of morbidity and frequently requires reintervention.

Surgical strategy to establish a dual-coronary system for the management of anomalous left coronary artery origin from the pulmonary artery.

BACKGROUND: Optimal repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) relies on the creation of a dual-coronary system. If the anomalous coronary arises at a long distance from the aorta, we use various coronary extension techniques to facilitate tension-free implantation. METHODS: Thirty patients underwent ALCAPA operations using direct coronary transfer (n = 11) or coronary extension techniques (n = 19). Surgical outcomes were analyzed. RESULTS: Median age and weight were 5.7 months (range, 46 days to 5.45 years) and 5.35 kg (range, 3.3 to 15.9 kg). Five patients had concomitant mitral annuloplasty. Mean cardiopulmonary bypass and ischemic times were 108 +/- 38 and 57 +/- 25 minutes. Two patients required intraoperative revision of the implantation. There were three hospital deaths (10%) and no late deaths. Follow-up echocardiograms demonstrated significant improvement postoperatively vs preoperatively in shortening fraction (35% +/- 2% vs 16% +/- 2%, p < 0.00001), ejection fraction (64% +/- 3% vs 32% +/- 4%, p < 0.00001), and mitral regurgitation (11% moderate vs 70% moderate or severe, p = 0.0002). Left ventricular end-diastolic dimension Z-score decreased from 9.1 +/- 0.9 to 1.2 +/- 0.5 (p < 0.00001). Both techniques were equally effective. Two patients underwent reoperation 1 and 12 years postoperatively (coronary artery bypass grafting, 1; mitral repair with coronary angioplasty, 1). Surviving patients remain asymptomatic (p < 0.00001). CONCLUSIONS: Dual-coronary system can be established in patients with ALCAPA. Coronary extension implantation techniques have acceptable operative mortality and excellent cardiac recovery and late survival. Although the rate of late coronary occlusion is low, continual ventricular or mitral dysfunction should trigger evaluation of persistent coronary compromise.

Treatment of status epilepticus in children.

Status epilepticus is a medical emergency that requires prompt recognition and urgent treatment. Successful management of status epilepticus in children depends upon implementation of a treatment protocol modified according to current practices as well as availability of antiepileptic medications, out of hospital emergency services and intensive-care facilities. We propose a treatment protocol for status epilepticus in children in the Kingdom of Saudi Arabia.