Expanding percutaneous treatment of mitral valve diseases: Transcatheter closure of mitral valve leaflet perforation.

Mitral valve perforation is a rare cause of mitral regurgitation. We present a case of a 16-year-old patient with mitral valve regurgitation after redo-cardiac surgery for recurrent subaortic stenosis. Transthoracic echocardiography revealed a mitral regurgitation with an eccentric jet causing a significant regurgitation documented by the presence of a convergence flow over the hole. This finding was corroborating by transesophageal echocardiography locating the perforation from the region of A2 scallop. Three-dimensional transesophageal echocardiography confirmed these findings and played a key role guiding the procedure. Typical approach is usually a cardiac surgical procedure based on repair the perforation, but the mitral orifice was successfully closed percutaneously using an Amplatzer Duct Occluder II (ADO II; Abbott Vascular, IL).

Cardiovascular adaptation to extrauterine life after intrauterine growth restriction.

Introduction The adaptive changes of the foetal heart in intrauterine growth restriction can persist postnatally. Data regarding its consequences for early circulatory adaptation to extrauterine life are scarce. The aim of this study was to assess cardiac morphometry and function in newborns with late-onset intrauterine growth restriction to test the hypothesis that intrauterine growth restriction causes cardiac shape and functional changes at birth. METHODS: A comprehensive echocardiographic study was performed in 25 neonates with intrauterine growth restriction and 25 adequate-for-gestational-age neonates. RESULTS: Compared with controls, neonates with intrauterine growth restriction had more globular ventricles, lower longitudinal tricuspid annular motion, and higher left stroke volume without differences in the heart rate. Neonates with intrauterine growth restriction also showed subclinical signs of diastolic dysfunction in the tissue Doppler imaging with lower values of early (e') diastolic annular peak velocities in the septal annulus. Finally, the Tei index in the tricuspid annulus was higher in the intrauterine growth restriction group. CONCLUSION: Neonates with history of intrauterine growth restriction showed cardiac remodelling and signs of systolic and diastolic dysfunction. Overall, there was a significant tendency to worse cardiac function results in the right heart. The adaptation to extrauterine life occurred with more globular hearts, higher stroke volumes but a similar heart rate compared to adequate-for-gestational-age neonates.

Propranolol as a treatment for multiple coronary artery micro-fistulas.

We present a case of congenital multiple coronary artery-left ventricle micro-fistulas, which were treated with propranolol disappearing within 6 months. She had a malformative syndrome associated with a chromosomal abnormality. The treatment for coronary artery fistula includes surgical ligation and transcatheter closure, but they are not indicated in congenital micro-fistulas. We propose propranolol as a treatment in this type of diffuse fistula.

Single coronary artery-right ventricular fistula.

We report a rare case of a male child aged 4 years and 5 months who was diagnosed with a coronary artery fistula and left single coronary artery. Pre-operative evaluation with echocardiography and selective angiography showed a dilated and tortuous single coronary artery draining into the right ventricular outflow tract. The coronary fistula was ligated. The post-operative and clinical courses were uneventful.

Double venous drainage in scimitar syndrome. Ideal anatomy for percutaneous complete cure.

Congenital venolobular or scimitar syndrome is a rare congenital cardiopulmonary anomaly consisting in a partial anomalous pulmonary venous drainage, lung hypoplasia, and anomalous systemic arterial supply to the lung. It can associate with other congenital disorders which will confer the clinical presentation and prognosis of these patients. In most of the cases, the therapeutic approach is partial, as anatomy allows only aberrant arterial embolisation. We present a 6-year-old girl with recurrent pulmonary infections, diagnosed as scimitar syndrome with double collector drainage to the inferior caval vein and left atrium, undergoing interventional catheterisation for complete correction of her disorder. The anomalous systemic artery supply was embolised and the anomalous venous drainage was occluded. The patient was asymptomatic during follow-up, which supports the interventional catheterisation approach as a valid therapeutic option in cases of scimitar syndrome with double venous drainage.

Defibrillation threshold decrease with the supradiaphragmatic extracardiac implantable cardioverter-defibrillator implantation technique.

Despite advances in implantable cardioverter-defibrillator (ICD) technology, the optimal ICD implantation technique for pediatric patients has not yet been established. One increasingly used option is totally extracardiac implantation. However, concern exists about the high defibrillation threshold (DFT) at the moment of implantation or during follow-up. We report the case of a 3-year-old boy with repetitive syncopal idiopathic ventricular tachycardia episodes treated with ICD implantation using the extracardiac technique. Changing device position from abdominal to a supradiaphragmatic, solved unsafe elevated discharge impedance and DFT during follow-up.

Percutaneous closure of a patent arterial duct in a newborn.

We report a successful percutaneous neonatal closure of haemodynamically significant patent arterial duct. The neonate was suffering multiorgan failure, and had complete atrioventricular block. Closure of the patent arterial duct, either with surgery or inhibitors of cyclooxygenase, was contraindicated. We inserted the Amplatzer Duct Occluder II, showing that percutaneous closure of a patent arterial duct is now feasible in critically ill neonates.

Subcutaneous Fat Necrosis of the Newborn: Report of Five Cases.

Subcutaneous fat necrosis of the newborn (SCFN) is a rare, self-limited disorder of the panniculus which appears in the first few weeks of life. SCFN generally follows an uncomplicated course. However, there are important complications for which the patient must be regularly monitored, including thrombocytopenia, hypoglycemia, hypertriglyceridemia, and most importantly, hypercalcemia. We report five infants with SCFN. All children were born at term. The onset of lesions was between 1 day and 20 days after birth. The back was the most frequent location. Birth hypoxia was the most frequent risk factor. Complications included hypercalcemia, hypoglycemia, and metastatic calcifications (disseminated subcutaneous calcifications, nephrocalcinosis, and myocardial calcifications). This study provides the first case of SCFN in the context of hypoxic encephalopathy and refractory septic shock that required extracorporeal membrane oxygenation (ECMO).

[Ductus arteriosus patency with stenting in critical pulmonary stenosis and pulmonary atresia with intact interventricular septum]. / Permeabilidad del conducto arterioso con stent en la estenosis pulmonar crítica y atresia pulmonar con septo interventricular intacto.

We report our experience with stenting of the ductus arteriosus in three neonates. Two patients had pulmonary atresia with an intact interventricular septum and one had critical pulmonary stenosis. Radiofrequency ablation was used to open the atretic pulmonary valve in two patients. In all three patients implanting a stent avoided the need for surgical creation of a Blalock-Taussig shunt. In two patients the procedure was scheduled as elective surgery, and in one it was done as an emergency procedure. Ductus arteriosus stenting is an alternative to palliative cardiac surgery.

[Aortic origin of the left pulmonary artery in an infant with Fallot's tetralogy]. / Origen aórtico de arteria pulmonar izquierda en neonato con tetralogía de Fallot.

We report the case of a male neonate who had a prenatal diagnosis of Fallot's tetralogy. He presented with respiratory distress during the second week of life. Chest x-ray showed an enlarged right ventricle and pulmonary edema. Echocardiography demonstrated characteristic features of Fallot's tetralogy. However, cardiac catheterization disclosed that, in this case of Fallot's tetralogy, the left pulmonary artery had an anomalous origin in the ascending aorta. At 23 days of age, the patient underwent total surgical correction of the defects, during which a direct anastomosis was employed without conduit placement. 10 days later, he was discharged. We present the results of a literature review of the epidemiological, physiopathological, clinical, diagnostic, and surgical characteristics of this rare condition.

[Percutaneous closure of ventricular septal defect with an Amplatzer device]. / Cierre percutáneo de la comunicación interventricular con dispositivo Amplatzer.

We present a series of 4 patients in whom a ventricular septal defect (VSD) was closed with an Amplatzer muscular VSD device during cardiac catheterization. In one patient with type I truncus arteriosus and subarterial VSD, closure of a wide apical defect was done to allow further surgical correction while avoiding left ventricular ventriculotomy. The second patient had congenitally corrected transposition of the great arteries with complete A-V block and a pacemaker implanted from birth. Our intention was unload the systemic anatomical right ventricle. The third patient had had 3 heart surgeries to correct a double outlet right ventricle. She had a residual ventricular septal defect which was large enough to cause heart failure. The last patient had an isolated muscular ventricular septal defect. Percutaneous closure was successful in all patients, and there were no complications. Percutaneous closure of the defect may be used as a primary procedure, before surgery, or as a complementary procedure after surgery.

Neonatal Ross-Konno operation and endocardial fibroelastosis resection after foetal percutaneous aortic valve balloon valvuloplasty: a complex approach to rescue the left heart.

We report a case of a patient who presented with aortic stenosis and a borderline left ventricle during foetal life. A balloon aortic valve valvuloplasty was performed in uterus, and in the postnatal period for relief of the left ventricular outflow tract obstruction followed by a Ross-Konno procedure with fibroelastosis resection. These successful interventions allowed left ventricular growth and the conversion to a biventricular circulation after a single-stage surgery.

[Surgical treatment of anomalous left pulmonary artery]. / Tratamiento quirúrgico del origen anómalo de la arteria pulmonar izquierda.

We report our experience with the surgical treatment of anomalous origin of the left pulmonary artery in eight children between 2004 and 2009. The congenital heart disease most frequently associated with this condition was patent ductus arteriosus. Surgery was carried out with extracorporeal circulation in five children, and without, in three. The anomalous pulmonary artery was divided and translocated to the main pulmonary artery. One patient died soon after surgery because of hemodynamic instability and another died later because of respiratory complications. The other patients progressed satisfactorily during follow-up: the reimplanted artery remained patent in all cases and respiratory symptoms improved. However, one patient required endoscopic treatment.

Enfermedad de Kawasaki / Malaltia de Kawasaki / Kawasaki´s disease

La malaltia de Kawasaki (MK) és una vasculitis sistèmica aguda d'etiologia desconeguda. El diagnòstic es basa en criteris clínics que inclouen febre, exantema, conjuntivitis, canvis en les extremitats, eritema de la mucosa oral i llavis, i adenopaties cervicals. No obstant això, aquests criteris tenen una sensibilitat i una especificitat baixes i, per tant, altres característiques clíniques i de laboratori poden ser útils per establir el diagnòstic, sobretot en els casos d'MK atípica o incompleta. El pronòstic depèn de l'extensió de l'afectació cardíaca; els aneurismes coronaris, que es de-sen volupen en el 20-25% dels pacients no tractats, poden provocar infart de miocardi o mort sobtada en l'edat adulta. El tractament amb altes dosis d'immunoglobulina intrave-nosa és eficaç per reduir el risc d'aneurismes coronaris en la majoria dels casos i és el tractament d'elecció. En aquesta revisió analitzem la clínica, l'epidemiologia i el tractament d'aquesta malaltia típica de l'edat pediàtrica

La enfermedad de Kawasaki (EK) es una vasculitis sistémica aguda de etiología desconocida. El diagnóstico se basa en criterios clínicos que incluyen fiebre, exantema, conjuntivitis, cambios en las extremidades, eritema de la mucosa oral y labios, y adenopatías cervicales. Sin embargo, estos criterios tienen una sensibilidad y una especificidad bajas y, por tanto, otras características clínicas y de laboratorio pueden ser útiles para establecer el diagnóstico, sobre todo en los casos de MK atípica o incompleta. El pronóstico depende de la extensión de la afectación cardiaca; los aneurismas coronarios, que se desarrollan en el 20-25% de los pacientes no tratados, pueden provocar infarto de miocardio o muerte súbita en la edad adulta. El tratamiento con altas dosis de inmunoglobulina intravenosa es eficaz para reducir el riesgo de aneurismas coronarios en la mayoría de los casos y es el tratamiento de elección. En esta revisión analizamos la clínica, la epidemiología y el tratamiento de esta enfermedad típica de la edad pediátrica (AU)

Kawasaki disease (MK) is an acute systemic vasculitis of unknown etiology. The diagnosis is based on clinical criteria that includes fever, rash, conjunctivitis, changes in the limbs, erythema of the oral mucosa and lips, and cervical lymphadenopathy. However, these criteria have a low sensitivity and specificity and, therefore, other clinical and laboratory features may be helpful in establishing the diagnosis, especially in cases of atypical or incomplete MK. The prognosis depends on the extent of heart involvement; coronary aneurysms, which develop in 20-25% of untreated patients can cause a heart attack or sudden death in adulthood. Treatment with high doses of intravenous immunoglobulin is effective to reduce the risk of coronary aneurysms in most cases and is the treatment of choice. In this review we analyze the symptoms, epidemiology and treatment of this disease, typical of paediatric patients (AU)

Complex aortic coarctation and PHACE syndrome.

In PHACE syndrome, the acronym PHACE stands for the association of posterior fossa malformations, cervicofacial hemangiomas, arterial anomalies, coarctation and eye anomalies. We report our findings in four patients with this syndrome, in whom it was characterized by complex aortic coarctation that required not only preoperative echocardiographic investigation, but also the use of techniques such as magnetic resonance imaging and angiography. Surgical treatment was also complex. Prognosis in this condition depends primarily on cardiovascular and cerebral artery complications associated with the syndrome.

Percutaneous treatment of atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus in infants under one year of age.

Amplatzer devices are used for the percutaneous closure of ostium secundum atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus. However, very little experience has been gained in using these devices in infants under 1 year of age. Between January 2001 and January 2008, 22 symptomatic infants aged under 1 year underwent percutaneous treatment: three had an ostium secundum atrial septal defect, 15 had patent ductus arteriosus, and four had a muscular ventricular septal defect. All the procedures were completed successfully. No immediate or medium-term complications were observed. Closure of these types of defect using an Amplatzer device in infants under 1 year of age, who would otherwise require surgery, is a safe and effective procedure.

Device migration in hybrid technique for apical muscular ventricular septal defects closure.

Muscular ventricular septal defects still require complex surgical procedures for their repair. We have used a hybrid approach for closure of these ventricular septal defects in patients needing open-heart surgery. It consists of the deployment of a ventricular septal occluder, as used in transcatheter procedures inside the defect under direct vision after cardiopulmonary bypass establishment. Through this paper, we report a case to illustrate a new and simple technique to avoid one of the most dramatic complications after this procedure: the migration of the closure device.

Respiratory infection in congenital cardiac disease. Hospitalizations in young children in Spain during 2004 and 2005: the CIVIC Epidemiologic Study.

OBJECTIVES: To evaluate the rate of hospitalization for acute respiratory tract infection in children less than 24 months with haemodynamically significant congenital cardiac disease, and to describe associated risk factors, preventive measures, aetiology, and clinical course. MATERIALS AND METHODS: We followed 760 subjects from October 2004 through April 2005 in an epidemiological, multicentric, observational, follow-up, prospective study involving 53 Spanish hospitals. RESULTS: Of our cohort, 79 patients (10.4%, 95% CI: 8.2%-12.6%) required a total of 105 admissions to hospital related to respiratory infections. The incidence rate was 21.4 new admissions per 1000 patients-months. Significant associated risk factors for hospitalization included, with odds ratios and 95% confidence intervals shown in parentheses: 22q11 deletion (8.2, 2.5-26.3), weight below the 10th centile (5.2, 1.6-17.4), previous respiratory disease (4.5, 2.3-8.6), incomplete immunoprophylaxis against respiratory syncytial virus (2.2, 1.2-3.9), trisomy 21 (2.1, 1.1-4.2), cardiopulmonary bypass (2.0, 1.1-3.4), and siblings aged less than 11 years old (1.7, 1.1-2.9). Bronchiolitis (51.4%), upper respiratory tract infections (25.7%), and pneumonia (20%) were the main diagnoses. An infectious agent was found in 37 cases (35.2%): respiratory syncytial virus in 25, Streptococcus pneumoniae in 5, and Haemophilus influenzae in 4. The odds ratio for hospitalization due to infection by the respiratory syncytial virus increases by 3.05 (95% CI: 2.14 to 4.35) in patients with incomplete prophylaxis. The median length of hospitalization was 7 days. In 18 patients (17.1%), the clinical course of respiratory infection was complicated and 2 died. CONCLUSIONS: Hospital admissions for respiratory infection in young children with haemodynamically significant congenital cardiac disease are mainly associated with non-cardiac conditions, which may be genetic, malnutrition, or respiratory, and to cardiopulmonary bypass. Respiratory syncytial virus was the most commonly identified infectious agent. Incomplete immunoprophylaxis against the virus increased the risk of hospitalization.