Springer Nature is making SARS-CoV-2 and COVID-19 research free. View research | View latest news | Sign up for updates

Abnormalities of breathing control and airway maintenance in infants and children as a cause of cor pulmonale

Summary

Respiratory control abnormalities may result in cor pulmonale. This report summarizes the clinical history, diagnostic evaluation, treatment, and outcome of 16 infants and children presenting with cor pulmonale subsequently found to be due to sleep-dependent hypoventilation. Eleven patients had cardiomegaly and electrocardiographic evidence of right ventricular hypertrophy (RVH) while 5 had only severe RVH or biventricular hypertrophy (BVH). Four infants with central hypoventilation syndrome (CHS)—absence of sleep-related ventilatory drive—had severe sleep-dependent asphyxia and resultant acute respiratory failure; all were ultimately treated with phrenic nerve pacing. One patient with alveolar hypoventilation syndrome (AHS)—a partial deficit in ventilatory drive during sleep—presented with severe pulmonary hypertension and ultimately died despite symptomatic relief with respiratory stimulants. Eleven patients presented with obstructive sleep apnea (OSA) and sleep-dependent asphyxia secondary to intermittent complete or to prolonged partial upper airway obstruction. Localized airway obstruction due to an anomalous innominate artery in 1 child was corrected by arteriopexy. Four children underwent adenotonsillectomy (T&A) with disappearance of symptoms in 1, clinical improvement in 2 and no clinical improvement in another. This unimproved patient and the 6 remaining OSA children improved dramatically after tracheostomy to bypass the sleep-dependent airway obstruction; none presently has evidence of cor pulmonale. In summary, early recognition and appropriate treatment of respiratory control disorders will improve sleep ventilation, eliminate asphyxia during sleep, and prevent the development of cor pulmonale.

This is a preview of subscription content, log in to check access.

References

  1. 1.

    Brouillette RT, Fernbach S, Hunt CE: Obstructive sleep apnea in infants and children.J Pediatr. 100:31–40, 1982

  2. 2.

    Brouillette RT, Thach BT: A neuromuscular mechanism maintaining extrathoracic airway patency.J Appl Physiol: Resp Environ Exercise Physiol 46:772–779, 1979

  3. 3.

    Eliaschar I, Lavie P, Halperin E, Gordon C, Alroy G: Sleep apneic episodes as indications for adenotonsillectomy.Arch Otolaryngol 106:492–496, 1980

  4. 4.

    Felman AH, Loughlin GM, Leftridge CA, Cassisi NJ: Upper airway obstruction during sleep in children.AJR 133:213–216, 1979

  5. 5.

    Fleming PJ, Cade D, Bryan MH, Bryan AC: Congenital central hypoventilation and sleep state.Pediatrics 66:425–428, 1980

  6. 6.

    Guilleminault C, Cummiskey J, Motta J: Chronic obstructive airflow disease and sleep studies.Am Rev Resp Dis 122:397–406, 1980

  7. 7.

    Hunt CE: Abnormal hypercarbic and hypoxic sleep arousal responses in near-miss SIDS infants.Pediatr Res 15:1462–1464, 1981

  8. 8.

    Hunt CE, Inwood RJ, Shannon DC: Respiratory and nonrespiratory effects of doxapram in congenital central hypoventilation syndrome.Am Rev Resp Dis 119:263–269, 1979

  9. 9.

    Hunt CE, Matalon SV, Thompson TR, Demuth S, Loew JM, Liu HM, Mastri A, Burke B: Central hypoventilation syndrome: Experience with bilateral phrenic nerve pacing in 3 neonates.Am Rev Resp Dis 118:23–28, 1978

  10. 10.

    Hunt CE, McCulloch K, Brouillette RT: Diminished hypoxic ventilatory responses in near-miss sudden infant death syndrome.J Appl Physiol: Resp Environ Exercise Physiol 50:1313–1317, 1981

  11. 11.

    Ilbawi MN, Hunt CE, DeLeon SY, Idriss FS: Diaphragmatic pacing in infants and children: Report of a simplified technique and review of experience.Ann Thorac Surg 31:61–65, 1982

  12. 12.

    Kravath RE, Pollak CP, Borowiecki B: Hypoventilation during sleep in children who have lymphoid airway obstruction treated by nasopharyngeal tube and T and A.Pediatrics 59:865–871, 1977

  13. 13.

    Mustard WT, Bayliss CE, Fearon B, Pelton D, Trusler GA: Tracheal compression by the innominate artery in children.Ann Thorac Surg 8:312–319, 1969

  14. 14.

    Orenstein DM, Boat TF, Stern RC, Doershuk CF, Light MS: Progesterone treatment of the obesity hypoventilation syndrome in a child.J Pediatr 90:477–479, 1977

  15. 15.

    Remmers JE, DeGroot WJ, Sauerland EK, Anch AM: Pathogenesis of upper airway occlusion during sleep.J Appl Physiol: Respir Environ Exercise Physiol 44:931–938, 1978

  16. 16.

    Richardson MA, Seid AB, Cotton RT, Benton C, Kramer M: Evaluation of tonsils and adenoids in sleep apnea syndrome.The Laryngoscope 90:1106–1110, 1980

  17. 17.

    Sondheimer HM, Keith JD: Electrocardiography and Vectorcardiography InHeart Disease in Infancy and Childhood, 3rd ed. (Keith JD, Rowe RD, and Vlad P eds). New York: MacMillan Publishing Co, 1978, p 51–65

  18. 18.

    Wilson SL, Thach BT, Brouillette RT, Abu-Osba YK: Upper airway patency in the human infant: Influence of airway pressure and posture.J Appl Physiol: Resp Environ Exercise Physiol 48:500–504, 1980A0946011 00011 CS-SPJRNPDF [HEADSUP]

Download references

Author information

Additional information

Supported in part by Children's Research Guild, Otho S. Sprague Foundation and NIH Grant RR-05475-14

This paper was part of the Ray C. Anderson Symposium

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Hunt, C.E., Brouillette, R.T. Abnormalities of breathing control and airway maintenance in infants and children as a cause of cor pulmonale. Pediatr Cardiol 3, 249–256 (1982). https://doi.org/10.1007/BF02240461

Download citation

Key words

  • Respiratory control abnormality
  • Airway maintenance
  • Cor pulmonale
  • Children