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The effect of long term ventilatory support on hemodynamics in children with spinal muscle atrophy (SMA) type II

Agneta Markström1, Gary Cohen, Miriam Katz-Salamon

  • 1Karolinska Institutet, Department of Clinical Sciences, Danderyd Hospital, National Respiratory Centre, Stockholm, Sweden. agnetamarkstrom@hotmail.com

Sleep Medicine
|January 13, 2010
PubMed

Insights

Bi-level Positive Airway Pressure (Bi-PAP) therapy in children with Spinal Muscular Atrophy (SMA) does not negatively impact heart rate or blood pressure when optimized. Proper mask fit is crucial for stable hemodynamics during Bi-PAP treatment.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Disorders
  • Cardiovascular Physiology

Background:

  • Bi-level Positive Airway Pressure (Bi-PAP) improves respiratory function in children with Spinal Muscular Atrophy (SMA) type II.
  • The impact of Bi-PAP on hemodynamic parameters like heart rate (HR) and blood pressure in these children remains largely unknown.
  • This study investigates the association between Bi-PAP during sleep and hemodynamic changes in children with SMA.

Purpose of the Study:

  • To determine if Bi-PAP therapy affects heart rate and blood pressure in children with SMA during sleep.
  • To assess the influence of optimal versus sub-optimal Bi-PAP settings on hemodynamic stability.
  • To evaluate the relationship between breathing efficiency and hemodynamic parameters under Bi-PAP support.

Main Methods:

  • Ten children (8-12 years) with SMA on long-term Bi-PAP underwent overnight polysomnography.
  • Heart rate (HR), electrocardiogram (ECG), respiratory movements, and blood gases were continuously monitored.
  • Blood pressure was estimated via pulse transit time (PTT); breathing efficiency was assessed using the phase angle between chest and abdominal movements.

Main Results:

  • Heart rate and pulse transit time during unsupported and optimal Bi-PAP breathing were comparable.
  • Blood gas levels (SaO2, TcO2, TcCO2) remained stable across breathing conditions.
  • Optimal Bi-PAP significantly improved breathing efficiency (phase angle decreased from 42 to 22 degrees).
  • Sub-optimal Bi-PAP with mask leakage led to increased variability in HR, PTT, and breathing effort.

Conclusions:

  • Optimized Bi-PAP therapy is hemodynamically safe for children with SMA.
  • Correct mask application and sealed fit are essential for maintaining stable hemodynamics.
  • Bi-PAP effectively enhances respiratory efficiency without adverse cardiovascular effects in pediatric SMA patients when properly managed.
Abstract

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