PubMed Health⌕ Search

Biomedical subjects

S Jiménez Casso

Publications and source records attributed to S Jiménez Casso.

8 recordsLinked to original sources

[Cardiac rhabdomyomas in tuberous sclerosis: clinical symptoms and course in 18 cases diagnosed in childhood].

OBJECTIVE: Tuberous sclerosis is a neurocutaneous syndrome with autosomal dominant inheritance. The reported incidence is 1:10000. Recent echocardiographic studies showed a 50% to 64% incidence of cardiac rhabdomyomas in patients with tuberous sclerosis. The objective is to assess the history of cardiac rhabdomyomas in infants and children with tuberous sclerosis. METHODS: From 1970 to 1998, 39 patients were diagnosticated of tuberous sclerosis. Cardiac rhabdomyomas were present in 18 of them, 9 girls and 9 boys. Ecocardiografic follow up ranged from 1 month to 14 years. RESULTS: Echocardiographic studies showed rhabdomyomas located in the interventricular septum, ventricles, right and left atrium. A newborn, had subaortic obstruction related to a tumor who precised surgery excision. Standard electrocardiogram showed different disturbances: premature auricular and ventricular contractions, conduccion disturbances and pre-excitation syndromes. Two patients, both neonates, had severe arrhythmias, one of them debuted with supraventricular tachycardia due to an accessory hidden left atrio-ventricular pathway who undergone successful radiofrequency ablation; and the other patient was diagnosticated prenatally of fetal complete heart block and precised permanent pacemaker at birth. After a follow up of 5.1+/-4.5 years, spontaneous regression of the tumors was shown in 12 of the 18 patients with complete resolution in two of them. All of them are asymptomatic nowadays. CONCLUSIONS: Our findings confirms that cardiac rhabdomyomas are most often a benign condition in which spontaneous regression is the rule and surgery is only recommended for patients with life threatening obstruction or refractory disrhythmias in the neonatal period when the tumor size is maximum.

Adolescent↗

[Coronary ischemia during radiofrequency ablation of left lateral accessory pathway in an infant].

Acute coronary ischaemic complications are uncommon following catheter ablation of accessory pathways. Only isolated cases have been previously reported in adult patients during ablation of left free-wall accessory pathways by retrograde or anterograde approach. We report a transient ischaemic complication after catheter ablation of a left free wall accessory pathway by transseptal approach in an infant.

Acute Disease↗

[Incessant ventricular tachycardia and myocardial hamartomas in childhood: long-term remission after surgical treatment].

Incessant ventricular tachycardia in children 2 years old or younger is usually caused by a well-localized myocardial lesion that has been termed myocardial hamartoma. Its major clinical manifestation is a rapid and potentially fatal tachyarrhythmia that does not respond to antiarrhythmic drugs. We report a patient who underwent successful surgical excision of the hamartoma and we describe the clinical features, pathologic findings and longterm follow-up.

Electrocardiography↗

[Congenital ectopic junctional tachycardia: long-term remission after cryoablation of the bundle of His].

Congenital junctional ectopic tachycardia is an uncommon and potentially fatal arrhythmia that does not usually respond to medical therapy. We report a two month old infant with severe ventricular failure. The diagnosis was confirmed by electrophysiologic study. She underwent endocardial cryoablation of the His bundle during extracorporeal circulation and a ventricle epicardial-lead pacemaker was implanted. Eight years later the patient is still symptom-free without needing medical treatment.

Bundle of His↗

[Aortic balloon valvuloplasty in critical aortic stenosis in the newborn and infants].

INTRODUCTION: Balloon valvuloplasty in neonates and small infants with critical aortic stenosis is a palliative procedure. The present report describes the results of the technique in our center. METHODS: From April 1993 to March 1995, six consecutive patients with critical aortic valve stenosis underwent catheter-balloon valvuloplasty. Their ages ranged from 2 to 120 days old (45.5 +/- 47.5 days). Four patients had associated lesions: 2 had coarctation of the aorta, 1 had ischemic dilated cardiomyopathy and 1 had endocardial fibroelastosis. Percutaneous femoral artery access was used in four cases and axillary artery dissection in two. RESULTS: The balloon-annulus diameter ratio was 0.92 +/- 0.12. The peak systolic ejection gradient decreased from 66.1 +/- 26.4 to 38 +/- 15.7 mmHg (p < 0.05) and the left ventricle systolic pressure decreased from 136.3 +/- 26.8 to 115 +/- 22.5 mmHg (p < 0.05). There were no mortalities related to the procedure. Both patients who had aortic coarctation developed aortic regurgitation and died after repairing of the coarctation. The patient with endocardial fibroelastosis died during an attempt to perform the Norwood operation (Stage I) and the other patient with ischemic dilated cardiomyopathy survived after cardiac transplantation. The remaining two patients with isolated aortic valve stenosis are currently asymptomatic. CONCLUSIONS: Catheter-balloon valvuloplasty is an effective procedure in the initial treatment of critical aortic stenosis and may be life saving.

Aortic Valve Stenosis↗