3 results
Long-term outcome of congenital aortic valve stenosis: predictors of reintervention
- Léa Hochstrasser, Patrick Ruchat, Nicole Sekarski, Michel Hurni, Ludwig K. von Segesser
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- Journal:
- Cardiology in the Young / Volume 25 / Issue 5 / June 2015
- Published online by Cambridge University Press:
- 01 July 2014, pp. 893-902
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Objectives: To evaluate long-term outcome of initial aortic valve intervention in a paediatric population with congenital aortic stenosis, and to determine risk factors associated with reintervention. Patients and methods: From 1985 to 2009, 77 patients with congenital aortic stenosis and a mean age of 5.8±5.6 years at diagnosis were followed up in our institution for 14.8±9.1 years. Results: First intervention was successful with 86% of patients having a residual peak aortic gradient <50 mmHg, and the proportion of patients with grade >1 regurgitation increased by 7%. Long-term survival after the first procedure was excellent, with 91% survival at 25 years. At a mean interval of 7.6±5.3 years, 30 patients required a reintervention (39%), mainly because of a recurrent aortic stenosis. Freedom from reintervention was 97, 89, 75, 53, and 42% at 1, 10, 15, 20, and 25 years, respectively. Predictors of reintervention were residual peak aortic gradient (p=0.0001), aortic regurgitation post-intervention >1 (p=0.02), prior balloon aortic valvuloplasty (p=0.04), and increased left ventricular posterior wall thickness (p=0.1). Conclusions: Aortic valve intervention is a safe and effective procedure for congenital aortic stenosis with excellent survival results. However, rate of reintervention is high and influenced by increased left ventricular posterior wall thickness pre-intervention, prior balloon valvuloplasty, higher residual peak systolic valve gradient, and more than mild regurgitation post-intervention. The study highlights that long-term follow-up is recommended for these patients.
Considerations for Prenatal Counselling of Patients with Cardiac Rhabdomyomas based on their Cardiac and Neurologic Outcomes
- Sabrina C. Degueldre, Priya Chockalingam, Yvan Mivelaz, Stefano Di Bernardo, Jean-Pierre Pfammatter, Catherine Barrea, Nicole Sekarski, Pierre-Yves Jeannet, Jean-Claude Fouron, Yvan Vial, Erik J. Meijboom
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- Journal:
- Cardiology in the Young / Volume 20 / Issue 1 / February 2010
- Published online by Cambridge University Press:
- 22 January 2010, pp. 18-24
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Cardiac rhabdomyomas are benign cardiac tumours with few cardiac complications, but with a known association to tuberous sclerosis that affects the neurologic outcome of the patients. We have analysed the long-term cardiac and neurological outcomes of patients with cardiac rhabdomyomas in order to allow comprehensive prenatal counselling, basing our findings on the records of all patients seen prenatally and postnatally with an echocardiographic diagnosis of cardiac rhabdomyoma encountered from August, 1982, to September, 2007. We analysed factors such as the number and the location of the tumours to establish their association with a diagnosis of tuberous sclerosis, predicting the cardiac and neurologic outcomes for the patients.
Cardiac complications include arrhythmias, obstruction of the ventricular outflow tracts, and secondary cardiogenic shock. Arrhythmias were encountered most often during the neonatal period, with supraventricular tachycardia being the commonest rhythm disturbance identified. No specific dimension or location of the cardiac rhabdomyomas predicted the disturbances of rhythm.
The importance of the diagnosis of tuberous sclerosis is exemplified by the neurodevelopmental complications, with four-fifths of the patients showing epilepsy, and two-thirds having delayed development. The presence of multiple cardiac tumours suggested a higher risk of being affected by tuberous sclerosis. The tumours generally regress after birth, and cardiac-related problems are rare after the perinatal period. Tuberous sclerosis and the associated neurodevelopmental complications dominate the clinical picture, and should form an important aspect of the prenatal counselling of parents.
Adequate left ventricular preparation allows for arterial switch despite late referral
- Antonio F. Corno, Michel Hurni, Maurice Payot, Nicole Sekarski, Piergiorgio Tozzi, Ludwig K. von Segesser
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- Journal:
- Cardiology in the Young / Volume 13 / Issue 1 / February 2003
- Published online by Cambridge University Press:
- 18 April 2005, pp. 49-52
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Objective: To evaluate the feasibility of the arterial switch for surgical repair of transposition, defined as the combination of concordant atrioventricular and discordant ventriculo-arterial connections, after late referral. Methods: From March 2000 to August 2001, six children underwent an arterial switch procedure following left ventricular preparation because of late referral. The mean age at referral was 8.3 months, with a range from 3 to 25 months, and mean body weight was 5.3 kg, with a range from 3.7 to 9.3 kg. The mean saturation of oxygen was 57%, with a range from 50 to 72%. Associated defects included a restrictive ventricular septal defect in three patients, aortic coarctation in one, and partially anomalous pulmonary venous connection in one. The mean interval between referral and the arterial switch procedure was 3.7 months, within a range from 1 to 7 months. A mean of 1.5 surgical procedures were undertaken to prepare the left ventricle, the most being 3 procedures, including combinations of creation of an inter-atrial communication in four patients, banding of the pulmonary trunk in five, and creation of a systemic-to-pulmonary arterial shunt in three. We evaluated left ventricle ejection and shortening fractions, left ventricular diastolic diameter and volume, right and left ventricular wall thicknesses, and the ratio of right to left ventricular values by echocardiography at referral, immediately before, and one week after the arterial switch procedure. Results: All children are alive and well, with a mean follow-up of 17 months, ranging from 9 to 26 months. Echocardiography showed a statistically significant decrease of the ratio between right and left ventricular wall thicknesses, from 1.33 ± 0.26 at referral to 0.79 ± 0.08 before the switch procedure (p < 0.005). Left ventricular function was adequate after arterial switch, with a mean ejection fraction of 79.3%, ranging from 66 to 87%, and a mean shortening fraction of 41.7%, ranging from 30 to 49%. Conclusions: Despite late referral, and initially inadequate left ventricular volume and mural thickness, children with transposition can successfully be treated with the arterial switch procedure, provided that the left ventricle is adequately prepared, using echocardiography to monitor left ventricular morphology and function.