Palliative Arterial Switch as First-line Treatment Before the Fontan Procedure in Patients With Single-ventricle Physiology and Subaortic Stenosis

There are several techniques for the palliative treatment of patients with single-ventricle physiology, ventriculoarterial discordance and subaortic stenosis. The Fontan procedure relies on optimal initial palliation to avoid the development of subaortic stenosis (as well as ventricular hypertrophy...

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Bibliographic Details
Published in:Revista española de cardiología (English ed.) 2013-07, Vol.66 (7), p.553-555
Main Authors: Gil-Jaurena, Juan Miguel, Zabala, Juan-Ignacio, Albert, Dimpna C., Castillo, Rafael, González, Mayte, Miró, Luis
Format: Article
Language:English
Subjects:
Aortic Stenosis, Subvalvular - surgery
Female
Follow-Up Studies
Fontan
Fontan Procedure - adverse effects
Fontan Procedure - methods
Glenn
Heart Defects, Congenital - surgery
Humans
Infant
Infant, Newborn
Male
Paliativo
Palliative
Palliative Care - methods
Single-ventricle
Switch
Transposición
Transposition
Univentricular
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Summary:There are several techniques for the palliative treatment of patients with single-ventricle physiology, ventriculoarterial discordance and subaortic stenosis. The Fontan procedure relies on optimal initial palliation to avoid the development of subaortic stenosis (as well as ventricular hypertrophy and diastolic dysfunction). We present seven patients with single-ventricle physiology, transposition of the great arteries and subaortic stenosis, with low systemic output and high pulmonary flow, aged 21 to 383 days (median, 75) and weighing between 3.4 and 9.6kg (median, 4.2). All were treated with a palliative arterial switch, thus “switching” their subaortic stenosis to subpulmonary stenosis. Six patients also underwent aortic arch surgery, 4 an atrial septectomy, and 1 a subaortic membrane resection. One patient died after surgery, another developed recoarctation, which was treated with an angioplasty, 3 patients reached the Glenn stage and 2 the Fontan stage. All had good ventricular function. A palliative switch is an effective initial treatment for single-ventricle physiology with transposition of the great arteries and subaortic stenosis. This complex initial technique produces good results and allows the patient to progress to the Glenn or Fontan stage. Para la paliación inicial de pacientes con fisiología univentricular, discordancia ventriculoarterial y estenosis subaórtica, se utilizan diversas técnicas. La cirugía tipo Fontan depende de una correcta paliación inicial que evite la aparición de estenosis subaórtica (además de hipertrofia ventricular y disfunción diastólica). Presentamos a 7 pacientes con fisiología univentricular, transposición y estenosis subaórtica, con bajo gasto sistémico e hiperaflujo pulmonar, de 21–383 (mediana, 75) días de edad y 3,4–9,6 (mediana, 4,2) kg de peso. Todos fueron tratados mediante switch arterial paliativo, «cambiando» su estenosis subaórtica por una estenosis subpulmonar. En 6 casos se asoció cirugía de arco aórtico; en 4, septectomía auricular y en 1, resección de membrana subaórtica. Un paciente falleció en el postoperatorio; otro sufrió recoartación, tratada mediante angioplastia; 3 pacientes han alcanzado el estadio de Glenn y 2, el de Fontan. La función ventricular es correcta en todos ellos. La fisiología de ventrículo único con transposición y estenosis subaórtica se puede tratar inicialmente mediante un switch paliativo. Esta compleja técnica inicial presenta buenos resultados y permite la progresión f
ISSN:1885-5857
1885-5857
DOI:10.1016/j.rec.2013.01.020