Pediatric Primary Pulmonary Hypertension Differential Diagnoses

Author: Stuart Berger, MD; Chief Editor: Michael R Bye, MD more...

Updated: Mar 29, 2011

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Diagnostic Considerations

Because many of the symptoms of idiopathic pulmonary artery hypertension (IPAH) are nonspecific and the disorder is relatively rare, the diagnosis may be somewhat difficult to make. Failure to make the diagnosis or significant delay in making the diagnosis delays treatment, and early therapy may be beneficial in a subset of children.

Go to Primary Pulmonary Hypertension and Persistent Newborn Pulmonary Hypertension for more complete information on these topics.

Other problems to consider include the following:

Pulmonary venous hypertension (due to left heart obstruction)
Upper airway obstruction
Diminished ventilatory drive
Chest wall abnormalities
Pulmonary thromboembolism
Exogenous substances (eg, anorexic agents, toxic rapeseed oil, cocaine)
Portal Hypertension
Pulmonary veno-occlusive disease

Differentials

Proceed to Workup

Contributor Information and Disclosures

Author

Stuart Berger, MD Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Specialty Editor Board

Girish D Sharma, MD Associate Professor of Pediatrics, Rush Medical College; Director, Section of Pediatric Pulmonology and Rush Cystic Fibrosis Center, Rush University Medical Center

Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Heidi Connolly, MD Associate Professor of Pediatrics and Psychiatry, University of Rochester School of Medicine and Dentistry; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center

Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

References

Haworth SG. Idiopathic pulmonary arterial hypertension in childhood. Cardiol Rev. Mar-Apr 2010;18(2):64-6. [Medline].
Atwood CW Jr, McCrory D, Garcia JG, Abman SH, Ahearn GS. Pulmonary artery hypertension and sleep-disordered breathing: ACCP evidence-based clinical practice guidelines. Chest. Jul 2004;126(1 Suppl):72S-77S. [Medline].
Barst R, Long W, Gersony W. Long-term vasodilator treatment improves survival in children with primary pulmonary hypertension. Cardiol Young. 1993;3 (S1):89.
Atz AM, Wessel DL. Sildenafil ameliorates effects of inhaled nitric oxide withdrawal. Anesthesiology. Jul 1999;91(1):307-10. [Medline].
Lammers AE, Burch M, Benden C, Elliott MJ, Rees P, Haworth SG, et al. Lung transplantation in children with idiopathic pulmonary arterial hypertension. Pediatr Pulmonol. Mar 2010;45(3):263-9. [Medline].

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