Ivan Malčić

Are we improving after 10 years of humanitarian paediatric cardiac assistance

BACKGROUND Paediatric cardiovascular services are frequently absent or poorly developed in many countries around the world. Our foundation made 83 trips in support of cardiovascular services between April 1993 and March 2003 to help alleviate this problem. In this study, we present an analysis of our results over these period of 10 years. METHODS We performed a review of all available records relating to the trips, including patient databases, audited financial statements, donated product inventory lists, lists of team members, and follow-up data from the host sites concerning the state of the patients treated. RESULTS We made 83 trips to 14 countries, 40 of these being in Central Europe, 5 in Eastern Europe, 10 in Caribbean, and Central America, 18 in South America, 9 in Asia, and 1 in the Middle East. In the first 5 years, we made 23, as opposed to 60 in the second 5 years, this difference being significant (p less than 0.01). The total number of primary operations performed over 10 years was 1,580. The number of procedures performed yearly increased over the two intervals from 97.0 plus or minus 32.7 to 219.0 plus or minus 41.7, p less than 0.002. The probability of survival between the periods increased from 84.6 to 93.3 per cent, and this was also significantly different (p less than 0.001). Overall, the rate of survival for the period of 10 years was 90.5 per cent. Moreover, the value of services donated to support each trip also differed significantly, decreasing from 105,900 dollars plus or minus 14,581 dollars for the first period to 54,617 dollars plus or minus 11,425 dollars for the second period (p less than 0.001). CONCLUSIONS Improving paediatric cardiac services in under-served countries requires significant financial and personnel commitments, but can produce reasonable outcomes.

Epidemiology of cardiomyopathies in children and adolescents: a retrospective study over the last 10 years

We conducted a retrospective study at the Department of Paediatric Cardiology of the University Hospital Centre Rebro, Zagreb, over the period from 1988 to 1998, so as to assess the epidemiology of childhood cardiomyopathies. The patients were categorized according to the guidelines of the Task Force on Cardiomyopathies of the World Health Organization and the International Society and Federation of Cardiology. We identified 121 infants, children and adolescents as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 patients examined in our outpatient clinics for paediatric cardiology. Of the patients, 50 were female (41.3%) and 71 were male (58.7%). The cardiomyopathy was of the dilated variant in 52 patients (42.9%), with 43 patients (35.5%) having hypertrophic cardiomyopathy, and 6 patients (4.8%) identified with restrictive cardiomyopathy. We encountered no patients with arrhythmogenic right ventricular cardiomyopathy. In nine patients (7.4%), it proved impossible to classify the cardiomyopathy. We placed 11 patients (9.0%) in the group of specific cardiomyopathies. Most of those with dilated cardiomyopathy had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4-2.47). There were no statistically significant differences in the incidences of dilated as compared to hypertrophic cardiomyopathy (Z 0.923, p = 0.1779), but we encountered a significantly lower occurrence of restrictive cardiomyopathy (Z 6.044, p < 0.001). Of those with hypertrophic cardiomyopathy, 15 patients (34.8%) had the asymmetric variant, while 28 patients (65.2%) exhibited the concentric form. During the period of follow-up, 10 patients died, 4 with dilated cardiomyopathy, 4 with hypertrophic cardiomyopathy, 1 with restrictive cardiomyopathy, and 1 with a specific cardiomyopathy. We encountered 12 (9.9%) patients who, besides cardiomyopathies, also suffered from neuromuscular disorders. Most of these had dilated cardiomyopathy. Mitochondrial disorders, in contrast, were more frequently found in patients with hypertrophic cardiomyopathy.

Intracardiac Juvenile Xanthogranuloma in a Newborn

Abstract. Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.

Treatment of right ventricle to pulmonary artery conduit stenosis in infants with hypoplastic left heart syndrome

OBJECTIVES To determine the incidence of right ventricle-to-pulmonary artery (RV-PA) conduit stenosis after the Norwood I operation in patients with hypoplastic left heart syndrome (HLHS), and to determine whether the treatment strategy of RV-PA conduit stenosis has an influence on interstage and overall survival. METHODS Ninety-six patients had a Norwood operation with RV-PA conduit between 2002 and 2011. Details of reoperations/interventions due to conduit obstruction prior to bidirectional superior cavopulmonary anastomosis (BSCPA) were collected. RESULTS Overall pre-BSCPA mortality was 17%, early mortality after Norwood, 6%. Early angiography was performed in 34 patients due to desaturation at a median of 8 days after the Norwood operation. Fifteen patients (16%) were diagnosed with RV-PA conduit stenosis that required treatment. The location of the conduit stenosis was significantly different in the patients with non-ringed (proximal) and the patients with ring-enforced conduit (distal), P = 0.004. In 6 patients, a surgical revision of the conduit was performed; 3 of them died prior to BSCPA. Another 6 patients had a stent implantation and 3 were treated with balloon dilatation followed by a BSCPA in the subsequent 2 weeks. All patients who were treated interventionally for RV-PA conduit obstruction had a successful BSCPA. Patients who received a surgical RV-PA conduit revision had a significantly higher interstage (P = 0.044) and overall mortality (P = 0.011) than those who received a stent or balloon dilatation of the stenosis followed by an early BSCPA. CONCLUSIONS RV-PA conduit obstruction after Norwood I procedure in patients with HLHS can be safely and effectively treated by stent implantation, balloon dilatation and early BSCPA. Surgical revision of the RV-PA conduit can be reserved for patients in whom an interventional approach fails, and an early BSCPA is not an option.

False aneurysm origination from the proximal anastomosis of a right ventricular to pulmonary artery shunt following staged repair of hypoplastic left heart syndrome

The Norwood I operation, including placement of a shunt from the right ventricle to the pulmonary arteries, has been adopted by many surgeons for neonates with hypoplastic left heart syndrome. A three-year-old male who had undergone the Norwood I operation, and the Glenn operation, presented with a cervical pulsating tumor prior to the operation for total cavopulmonary connection. At the Glenn operation, the right ventricle to the pulmonary artery shunt was closed with a clip proximally, and the distal part was resected. Following the Glenn operation, the child had had recurrent deep sternal infections caused by Serratia marcescens. Cardiac catheterization showed a false aneurysm from the proximal shunt anastomosis. The bleeding after resternotomy was managed by initiating cardiopulmonary bypass via the groin vessels. Cerebral air embolies were prevented by systemic application of potassium, to achieve cardioplegic arrest during chest opening. The shunt was removed and the defect was closed. After the shunt was confirmed to be free from infection, a total cavopulmonary connection was performed after three days postoperatively. The case illustrates the management of retrosternal aneurysms during resternotomy in children.

A novel de novo TBX5 mutation in a patient with Holt–Oram syndrome leading to a dramatically reduced biological function

The Holt–Oram syndrome (HOS) is an autosomal dominant disorder affecting 1/100.000 live births. It is defined by upper limb anomalies and congenital heart defects with variable severity. We describe a dramatic phenotype of a male, 15‐month‐old patient being investigated for strict diagnostic criteria of HOS.

Huge Coronary Fistula From the Left Anterior Descending Coronary Artery to the Right Ventricle in a Newborn: A Rare Congenital Anomaly

We present a case of a neonate with a huge congenital fistula from the left anterior descending coronary artery to the right ventricle and additional heart defects. The circumflex coronary artery was supplied by the right coronary artery. The fistula was closed surgically at both ends.

Evaluation of paediatric cardiosurgical model in Croatia by using the Aristotle basic complexity score and the risk adjustment for congenital cardiac surgery-1 method

OBJECTIVE The Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were developed and used to compare outcomes of congenital cardiac surgery. Both methods were used to compare results of procedures performed on our patients in Croatian cardiosurgical centres and results of procedures were taken abroad. METHODS The study population consisted of all patients with congenital cardiac disease born to Croatian residents between 1 October, 2002 and 1 October, 2007 undergoing a cardiovascular operation during this period. RESULTS Of the 556 operations, the Aristotle basic complexity score could be assigned to 553 operations and the risk adjustment in congenital cardiac surgery-1 method to 536 operations. Procedures were performed in two institutions in Croatia and seven institutions abroad. The average complexity for cardiac procedures performed in Croatia was significantly lower. With both systems, along with the increase in complexity, there is also an increase in mortality before discharge and postoperative length of stay. Only after the adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications. CONCLUSION Both, the Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were predictive of in-hospital mortality as well as prolonged postoperative length to stay, and can be used as a tool in our country to evaluate a cardiosurgical model and recognise potential problems.

Ostial patch plasty of an aberrant right coronary artery in a symptomatic teenager.

An otherwise healthy 15-year-old girl presented with a history of transient myocardial ischemia. At the time, troponin levels were elevated to 20 microg/L. A myocardial perfusion scintigraphy evaluation revealed a minor scarring with an irreversible inferior defect at rest and at exercise. On admission, the patient was free from symptoms. Transthoracic echocardiography and computer tomography examinations revealed an aberrant origin of the right coronary artery (RCA) from the left sinus of Valsalva, just below the commissure. Repair was accomplished by incision into the RCA and patch plasty. An anomalous aortic origin of a coronary artery from the incorrect coronary sinus of Valsalva is a rare congenital cardiac defect that is associated with an increased risk of sudden death and cardiac morbidity; however, ischemia can occur even when the patient remains asymptomatic. Although this anomalous artery often shares a common orifice with the other coronary artery, it did not in the reported case. In conclusion, because of the high risk of sudden cardiac death, aggressive surgical management is indicated in symptomatic patients.

AB0998 Effects of Rituximab Therapy in 9 Children with Refractory Autoimmune Diseases (Systemic Lupus Erythematosus and ANCA Positive Vasculitis) – Single Centre Expirience

Background There are only few reports of rituximab (RTX) effects in children with autoimmune diseases refractory to conventional therapy protocols. Objectives Authors present their work on the effects of RTX therapy in chidren with systemic lupus erythematosus (SLE) + lupus nephritis (LN) and ANCA positive vasulitis, refractory to standard therapy. Methods Retrospective chart rewiev of all patients treated with RTX at the Departement of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, during the period 2009–2014. Results Nine children were treated with RTX: 6 with SLE + LN (3 boys, 3 girls) and 3 with ANCA positive vasulitis (3 girls). Median age of patients at the disease onset was 11.6 years (5 – 15 years). Conventional therapy included methylprednisolon, cyclophosphamide (CYC), mycophenolate mofetil, azathyoprine, in some cases plasmapheresis. In all cases RTX was introduced due to ineffectivness of conventional therapy in achiving the remission of autoimune disease. Median time between the beginnig of conventional therapy and introduction of RTX was 10.3 months (1 – 48 months). In 8 cases RTX (750 mg/m2, two doses, two weeks apart) was combined with mini pulses of CYC (350 mg/m2), in 1 case RTX was applied in dose of 375 mg/m2 per week, for 4 weeks. After RTX introduction complete, prolonged remission was achived in 6 children. In 2 patients relapses of disease required repeating of RTX therapy. One patient (girl (5 yrs) with ANCA possitive vasculitis) died due to initially severe presentation at disease onset and fulminant, progressive multiorgan failure despite all conventional therapy and early introduction of RTX. Conclusions Complete or partial remission after RTX introduction was achived in 8/9 (88,8%) of our patients with severe autoimmune diseases. To our expirience, RTX represents powerful tool in controlling SLE + LN and ANCA positive vasculitis refractory to conventional therapy protocols. References Yolanda Braun-Moscovici, Yonatan Butbul-Aviel, Ludmila Guralnik et.al. Rituximab: rescue therapy in life-threatening complications of refractory autoimmmune diseases: a single centre expirience. Rheumatol Int (2013) 33:1495-1504. RJ Davies, SR Sangle, NP Jordan et.al. Rituximab in the treatment of resistant lupus nephritis: therapy failure in rapidly progressive crescent lupus nephritis. Lupus (2013) 22, 574-582. D Duxbury, C. Combescure, C Chizzolini. Rituximab in systemic lupus erythematosus: an updated systemic review and meta-analysis. Lupus (2013) 22, 1489-1503. Marc Weidenbusch, Christoph Rommele, Angelika Schrottle, Hnas-Joachim Anders. Beyond the LUNAR trial. Efficacy of rituximab in refractory lupus nephritis. Nephrol Dial Transplant (2013) 28: 106-111. Maria Trachana, Artemis Koutsonikoli et.al. Safety and efficacy of Rituximab in refractory pediatric systemic lupus erythematosus nephritis: a single centre expirience of Northern Greece. Rheumatol Int (2013) 33:809-813. Disclosure of Interest None declared