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Vascular tortuosity syndrome is a rare genetic disorder that causes tortuosity and stenosis of the pulmonary, systemic and / or coronary circulations. As a result of treatment of pulmonary stenosis, symptoms of pulmonary edema, known as lung reperfusion syndrome, may occur. The case is presented of an adolescent patient with vascular tortuosity syndrome who presented with a pulmonary reperfusion syndrome after multiple stent implants in the left pulmonary artery. After the procedure, the patient immediately developed an acute pulmonary edema with severe clinical deterioration, which required assistance with extracorporeal membrane oxygenation for recovery.

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Plastic bronchitis is a rare complication in which bronchial casts of lymphatic origin develop in the tracheobronchial tree and cause airway obstruction. The main feature is expectoration of bronchial casts. This condition usually occurs in the postoperative period after the Fontan operation for congenital heart disease. Treatment modalities include mucolytics and chest physiotherapy, and in the most severe cases bronchoscopy to remove the casts and aerosolized urokinase or r-TPA. We describe a 12-year-old boy with pulmonary atresia with intact ventricular septum and severely hypoplastic right ventricle who underwent a modified Fontan operation at the age of 7 years. At the age of 12 years the patient started to present frequent episodes of coughing, dyspnea, and desaturation followed by difficult emission of white bronchial casts. An extracardiac Fontan conversion was performed to improve hemodynamics and symptoms. During the postoperative period frequent episodes of airway obstruction required bronchoscopy and finally improved with administration of aerosolized urokinase.

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Cardiac hydatid cyst is an uncommon disease, especially in children. The interventricular septum region is the most frequent localization. The cysts usually involve other organs making the etiologic diagnosis easier. Our young patient presented a single hydatid cyst. The etiologic diagnosis was difficult due to the negativity of the serologic tests and there were no other organs affected. Surgical ablation of the cyst was performed and histopathological analysis achieved.

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INTRODUCTION AND OBJECTIVES: Fetal cardiac tumors are rare and have a different histology than in adults: in the fetus, rhabdomyoma is more prevalent, but myxoma has not been described. We report our experience with nine fetuses with prenatally diagnosed primary cardiac tumours. METHODS: This is an observational and descriptive study of fetuses investigated because of the prenatal and echographic diagnosis of heart tumors. RESULTS: There were nine fetuses with thirteen cardiac masses among more than 700 fetal echocardiographic studies performed by pediatric cardiologists. Histology was available in four of them; three rhabdomyomas and once cavernous hemangioma. In one of the cases, the parents elected to interrupt the pregnancy; three patients died in the neonatal period (two as a direct consequence of the tumors and one due to sepsis) and we have no histological information regarding any of the other five fetuses. The subjects who survived beyond the neonatal period are mostly doing well. However, one developed tuberous sclerosis and another developed a hypoplastic left heart syndrome, perhaps due to the massive restriction of flow across the foramen ovale. The tumors in the other cases were well tolerated and hemodynamic or arrhythmic consequences were minimal of absent. No women with risk factors such as tuberous sclerosis were included in our study. CONCLUSIONS: Fetal cardiac tumors are rare and display a different histology. We recommend a conservative approach to treatment.

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BACKGROUND: Fetal supraventricular tachycardia is an infrequent and difficult to manage disease. Several therapeutic approaches have been proposed. We report our experience in its diagnosis and management. OBJECTIVES: Evaluation of the clinical features of fetal supraventricular tachycardia and efficacy of maternal and fetal medications in use for treatment. Immediate neonatal evolution is commented on. A therapeutic schema is proposed. PATIENTS: Nineteen fetuses with echocardiographically (M-mode and/or Doppler two dimensionally oriented) supraventricular tachycardia were diagnosed. RESULTS: Mean gestational age was 32 weeks; 8 fetuses had developed hydrops; 3 patients had dilated cardiomyopathy (two of them were hydropic). Two cases were prenatally diagnosed as atrial flutter (both of them without hydrops). Sixteen fetuses received transplacentary treatment: 8 with digital, 6 more associated with flecainide, direct administration of amiodarone to the fetus was added in two cases. Three patients died during follow-up (one postnatally, at third week of life), all of them had developed hydrops; arrhythmia characteristics were unmodified in three and control of cardiac rhythm was achieved in ten cases. CONCLUSIONS: In the majority of cases there are no cardiac malformations associated. It is possible to control the tachycardia, even if hydrops is present. It is not indicated to abbreviate the gestation. First election drugs are maternal digoxin and flecainide, they do not produce significant fetal or maternal secondary effects. Occasionally it is necessary to add or to change to other drugs or to propose more aggressive approaches.

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The case of a gestant diagnosed fetal arrhythmia in the 35th week of gestation is presented. A fetal echocardiography indicates an aneurysm of the interauricular septum and a fetal supraventricular tachycardia. Also is obtained an ambulatory 24-hour Holter electrocardiograph of the gestant and fetus rhythm. The register makes evident a gestant supraventricular tachycardia and as well a fetal supraventricular tachycardia, occurring in both without clinical company. Following the birth, the tachycardia in the newborn is identified to be automatic and incessant, requiring digital for its control. In postpartum registers of the mother no significant arrhythmia of any type is observed. We conclude that the ambulatory monitoring with a Holter electrocardiograph could be a good method of diagnosis and follow-up of fetal and gestant arrhythmias.

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Between february and december 1990, we attempted percutaneous closure of patent ductus arteriosus (PDA) in 26 patients and in one ostium secundum atrial septal defect (ASD). One of the PDA was right sided with right aortic arch and two were post surgical leaks. We used the "USCI-Rashkind PDA double disc occluder" and the newly designed "Lock Clamshell" occluder for the ASD. Twenty seven patients (20 females and 7 males), aged 1.3 to 16 years (M = 5.6) and weighing 9.5 to 56 kg (M = 21.3), were studied. Diagnosis was made clinically with the aid of Doppler colour flow echocardiography, with follow-up studies at 24 hours, 6 months and 1 year after the procedure. Diameter of the ductus varied from 1.6 to 8.2 mm (M = 2.6). Successful implant of the device was achieved in 25 cases (95%), of which 18 (72%) were totally occluded at 24 hours and 21 (84%) after 6 months, remaining small residual leaks in four (16%). In only one patient total occlusion was demonstrated 1 year after device implantation. We achieved 22 (88%) total occlusions. One case was not possible to occlude, due to its large size. Angiocardiography demonstrated adequate anatomy in 18, and difficult in 8 patients. Twelve and 17 mm devices were used in 16 and 9 patients, respectively. Successful second implant with subsequent total occlusion was achieved in one patient with a previous moderate residual leak. The ASD was central and single, with a diameter of 20 mm. Immediate total occlusion was achieved. We conclude that percutaneous PDA closure is a safe and effective procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

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We describe a patient with angiographic findings of a left superior persistent vena cava draining in the left atrium with absence of the coronary sinus' roof and coexisting with Holmes' heart. The diagnosis can be made by selective angiography in the left atrium, pulmonary vein or at the left superior vena cava. We want to emphasize the importance of knowing this sort of anomaly before surgical treatment.

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