[Brain abscess revealing trilogy of Fallot in a child: about a case at the University Teaching Hospital of Parakou, Benin]. / Abcès cérébraux révélant une trilogie de Fallot chez un enfant: à propos d'un cas au CHU de Parakou, Bénin.

Brain abscesses can cause significant morbidity in patients with cyanogen heart disease. In countries with limited resources treatment, it is difficult and prognosis is guarded. Here we report a case of brain abscesses revealing a rare form of cyanogen heart disease, the trilogy of Fallot, in Parakou in the north of Benin. The study involved a 9-year old boy, referred to a primary hospital for left hemiparesis. Interview and physical examination revealed symptoms evolving for two months including intense headaches, fever, vomiting and functional impairment of the left hemi-corps, altered general state, generalized cyanosis, left hemiparesis, pulmonary systolic murmur. Brain CT scan showed abscesses in the right parietal region and in the left temporal region as well as hydrocephalus. Doppler echocardiography showed stiff pulmonary stenosis, atrial septal defects and right ventricular hypertrophy. Antibiotic therapy including ceftriaxone, gentamicin and metronidazole was started in emergency. Indication for surgical intervention included trepano-puncture but this could not be performed due to rapid unfavorable outcome. Brain abscesses are a common complication of cyanogen heart disease. Outcome is fatal in the absence of adequate management, hence the role of diagnosis and early treatment of these heart diseases.

Pentology of Fallot's presenting with complete heart block.

A 17-year-old male patient presented with cyanosis, repeated squatting since childhood and haemoptysis since the past 1 month. He had central cyanosis with clubbing. Cardiovasular examination revealed ejection systolic murmur in the pulmonary area with single S2. ECG showed right ventricular hypertrophy (RVH) with right atrial enlargement and first-degree heart block. Two-dimensional echo showed ventricular septal defect, overriding aorta, RVH, right ventricular enlargement (RVE) and right atrial enlargement with infundibular and valvular pulmonary stenosis and 1.9 cm ostium secondum atrial septal defect. There was no evidence of atrioventricular canal defect. The patient was diagnosed with pentology of Fallots. Follow-up ECG showed complete heart block (CHB) that again reverted to first-degree heart block. A diagnosis of pentology of Fallot with intermittent CHB was made with an awake heart rate of 50/min. This case report shows association of CHB with tetralogy of Fallot.

Actualización 2013 en cardiopatías congénitas, cardiología clínica e insuficiencia cardiaca y trasplante / 2013 Update on Congenital Heart Disease, Clinical Cardiology, Heart Failure, and Heart Transplant

En este artículo se presentan las novedades más relevantes de 2013 en tres áreas clave de la cardiología: cardiopatías congénitas, cardiología clínica e insuficiencia cardiaca y trasplante. En cardiopatías congénitas se han revisado las aportaciones relacionadas con la muerte súbita del adulto con cardiopatía congénita, la importancia de algunos parámetros ecocardiográficos en la valoración del ventrículo derecho sistémico, los problemas del paciente con tetralogía de Fallot corregida e indicación de sustitución valvular pulmonar y la confirmación del papel que algunos factores tienen en la selección del candidato a cirugía de Fontan. Entre las novedades del área de cardiología clínica, están el documento para el correcto diagnóstico de las miocardiopatías elaborado por un grupo de trabajo europeo, estudios de coste-efectividad sobre implante percutáneo de prótesis aórticas, un consenso sobre el manejo de la disección de aorta tipo B y una guía de la enfermedad valvular aórtica y de aorta torácica ascendente. En insuficiencia cardiaca y trasplante, las novedades más importantes son las nuevas guías estadounidenses de insuficiencia cardiaca, los avances terapéuticos en la insuficiencia cardiaca aguda (serelaxina), el manejo de las comorbilidades como el déficit de hierro, la evaluación del riesgo con los nuevos biomarcadores y los avances en asistencia ventricular mecánica (AU)

This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters in assessing the systemic right ventricle, problems in patients with repaired tetralogy of Fallot and indication for pulmonary valve replacement, and confirmation of the role of specific factors in the selection of candidates for Fontan surgery. The most recent publications in clinical cardiology include a study by a European working group on correct diagnostic work-up in cardiomyopathies, studies on the cost-effectiveness of percutaneous aortic valve implantation, a consensus document on the management of type B aortic dissection, and guidelines on aortic valve and ascending aortic disease. The most noteworthy developments in heart failure and transplantation include new American guidelines on heart failure, therapeutic advances in acute heart failure (serelaxin), the management of comorbidities such as iron deficiency, risk assessment using new biomarkers, and advances in ventricular assist devices (AU)

Failure of neuraxial anaesthesia in a patient with Velocardiofacial syndrome.

Velocardiofacial or 22q11 deletion syndrome is a genetic condition caused by deletion 22q11, the deletion of a small segment of the long arm of chromosome 22. To our knowledge this is the first case report of a woman with Velocardiofacial syndrome presenting in late pregnancy for caesarean delivery. She had undergone a Tetralogy of Fallot repair as an infant and had residual pulmonary regurgitation. In addition examination revealed micrognathia and scoliosis. Neuraxial anaesthesia was unsuccessful and subsequent conversion to general anaesthesia was necessary despite concerns regarding her facial abnormalities, pulmonary regurgitation and mild intellectual impairment.

Acute-on-chronic liver failure due to thiamazole in a patient with hyperthyroidism and trilogy of Fallot: case report.

BACKGROUND: Thiamazole is a widely used antithyroid agent that has been approved for the treatment of hyperthyroidism. Although thiamazole-induced hepatotoxicity is a main side effect, it may progress to liver failure in a very few cases. CASE PRESENTATION: We described a 24-year-old patient with hyperthyroidism and trilogy of Fallot, who developed liver failure due to thiamazole. Liver biopsy showed intrahepatic cholestasis, mild inflammatory infiltrates, as well as significant fibrosis, indicating both acute and chronic liver injuries. Although a series of potent therapies were given, the patient deceased due to severe liver decompensation. CONCLUSIONS: This case suggests that thiamazole-induced hepatotoxicity in the setting of advanced fibrosis increases the risk of poor outcome. Regular liver function monitoring during thiamazole therapy is therefore important.

[Sudden transformation of pulmonary stenosis to trilogy of Fallot in a 65 year old patient]. / Transformation brutale d'un rétrécissement pulmonaire en trilogie de Fallot à 65 ans. Traitement par cathétérisme interventionnel.

The authors report the case of a 64 year old woman with typical valvular pulmonary stenosis in whom spontaneous and sudden reopening of the foramen ovale resulted in cyanosis. Transthoracic echocardiography with injection of contrast provides a complete diagnosis: valvular pulmonary stenosis with a mean pressure gradient of 83 mmHg and massive right-to-left interatrial shunt. The malformation was treated by interventional catheterisation in a two-stage procedure: pulmonary valvuloplasty followed by closure of the foramen ovale because of the persistence of a right-to-left interatrial shunt. The functional improvement was followed by the appearance of effort angina. Coronary angiography showed single vessel disease of the left anterior descending artery treated by stenting. The long-term outcome was satisfactory.

[Surgical treatment of atrial septal defects and associated congenital heart diseases]. / Khirurgicheskoe lechenie pri defekte mezhpredserdnoi peregorodki i sochetaiushchikhsia s nim porokakh serdtsa.

The results of radical correction of interatrial septum defects and heart diseases associated with it were analyzed after treatment of 502 patients at the age from 5 to 48 years. In most cases (58.4%) the patients were adults. The isolated defect of the interatrial septum was diagnosed in 377 patients. Correction of the defect was performed in 82 patients (16.3%) under mild hypothermia and 420 patients (83.6%) were operated upon under conditions of artificial circulation. General lethality was 7.7%.

[The animal experiments and clinical trial of impeller pump used as left ventricular or biventricular assist device].

The authors' impeller pump as a left ventricular or biventricular assist device was evaluated in animal experiments and clinical trial. Three calves survived over 6 weeks in left ventricular assist experiments and neither blood damage nor organ dysfunction of the animals was detected. In clinical trial, the pump supported both the greater and lesser circulation of a 6-year-old child with trilogy of Fallot and low output syndrome after open heart surgery. In 43 hours the pump worked properly. The pump weighing 240 g can perform either in pulsatile or in nonpulsatile mode. In pulsatile pumping, the impeller changes its rotating speed periodically by introducing a square wave form voltage into the motor coil.

[Surgical treatment of trilogy of Fallot in 42 adults].

From 1984 to 1989 corrective operation circulation was performed under extracorporeal in 42 adults with trilogy of Fallot (6.2% of total intracardial operations under direct vision in patients with congenital heart disease in our hospital). The obstruction was relieved by resection of the hypertrophial and sclerotic muscle trabeculae of the Rt. ventricular outflow tract and pulmonary valvotomy and patching with a piece of artificial blood vessel lined internally by auto-pericardium so that a bougie (more than 1.6cm) could be passed through. The Rt. ventricular pressure was reduced satisfactorily (PRV/PLV < 0.75). There was one operative death.

[Surgical treatment of trilogy of Fallot].

Two hundred and thirty-four patients with trilogy of Fallot including 102 cases of severe type were operated in our hospital in the last 15 years. Right ventricular outflow patching was done in 117 cases, Devega tricuspid annuloplasty in 7 cases. Total operative mortality rate was 2.6%. The pathological anatomy and the surgical treatment of the severe type cases were discussed.

[Postponed closure of the sternum after operations on the heart with extracorporeal circulation]. / Otstrochennoe zakrytie grudiny pri operatsiiakh na serdtse s iskusstvennym krovoobrashcheniem.

Data on postponed closure of the sternum in 3 patients who were operated on with extracorporeal circulation are discussed. Various clinical and technical aspects of the applied method are considered. On the basis of their personal experience and the literature data, the authors discuss the causes of the development of acute heart dilatation in operations with extracorporeal circulation and, with due regard for this, substantiate the indications for the use of the method. Postponed closure of the sternum is a promising method of treatment of critical hemodynamic disorders which occur at the stage of closure of the sternotomy and are characterized by the picture of cardiac tamponade.

Ultramicroscopic morphometric, biochemical and histophotometric investigations of myocardial biopsies taken from Fallot patients before and after cardioplegia with cold Kirsch's solution.

Biopsies taken from the myocardium of 5 patients with Morbus Fallot and from 1 patient with ventricle septum defect were investigated with a combination of morphometric, biochemical and histophotometric techniques in order to study the cardioprotective effect of cold Kirsch's solution. At the final phase of cardioplegia the cardiomyocytes reveal the following alterations: The volume densities of mitochondria, of their degenerated areas and that of cytoplasmic vacuoles show a significant increase whereas that of myofilaments decreases. Cristae and matrix mitochondriales, however, show only moderate alterations without statistical significance. Biochemically the total ATP-concentration and creatine phosphate (CP)-concentration were more or less diminished, in most of the cases the activity of the myosin ATPase was increased, that of the creatine phosphate kinase (CPK) diminished. Compared with the biochemical estimations of the ATPase activity, its histophotometric estimations yielded corresponding results in 2 of 4 cases. In general our findings confirm the cardioprotective effect of Kirsch's solution. The combination of methods used gives more reliable results than one technique alone.