eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Pulmonary Hypertension, Idiopathic: Follow-up

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
Contributor Information and Disclosures

Updated: Jul 21, 2008

Follow-up

Further Inpatient Care

  • Patients with idiopathic pulmonary artery hypertension (IPAH) may require inpatient care for any intercurrent illness that might result in significant hypoxemia or decreased cardiac output.
  • Also, patients with IPAH on continuous intravenous prostacyclin may have intravenous line–related complications requiring intravenous antibiotics and removal/replacement of their central venous access line.

Further Outpatient Care

  • Patients are observed on an outpatient basis with a regular, but somewhat variable, schedule.
  • Follow-up echocardiography is recommended on a variable schedule, depending on the degree of pulmonary hypertension and the clinical status of the patient.
  • In addition, follow-up cardiac catheterization and drug testing are also recommended. The latter can be performed in an outpatient setting, and its frequency depends on the clinical circumstances.

Inpatient & Outpatient Medications

  • The usual regimen for patients with IPAH includes warfarin (Coumadin), digoxin, and vasodilators, such as nifedipine, intravenous prostacyclin, or both.
  • Some patients are on continuous or nighttime supplemental oxygen therapy.

Deterrence/Prevention

  • Because the etiology of this disease is unknown, no methods of prevention are known.
  • Although the disease cannot be prevented, patients with moderate or severe IPAH are instructed to restrict their activity such that intense exercise is avoided. In addition other risks that might be associated with low cardiac output/pulmonary hypertensive crisis should be avoided.

Complications

  • Complications in patients with IPAH also are not uncommon. Patients who are treated with intravenous prostacyclin typically have line-related complications, including sepsis. The central line also commonly needs replacement.
  • Patients with severe IPAH are at risk of pulmonary hypertensive crises that can result in arrhythmias, syncope, and/or sudden cardiac death.

Prognosis

  • Prognosis varies and represents a significant improvement compared with previous years.
  • Children who respond to short-term drug testing have a 5-year survival rate of 90%, whereas children who do not initially respond have a 5-year survival rate of 33%. Further follow-up studies suggest this number to be much higher. Studies of newer medications, as well as combination medications, suggest a much improved longer-term prognosis, even for the acute nonresponder group, with some studies suggesting as high as an 80% 5-year survival rate.
  • For those children who truly do not respond to long-term therapy and who are symptomatic, lung transplantation should be considered. Although transplantation is also a palliative therapy, trading one disease for another, it often results in improvement of symptoms and quality of life. Living lobar lung donation may offer some benefits as compared to traditional cadaveric transplantation.

Patient Education

  • Patients must be educated with regard to central line care, signs and symptoms of line infection, and signs and symptoms of deteriorating condition.
  • In addition, patients on continuous intravenous prostacyclin develop tachyphylaxis and require interval dose increases. Families must learn the proper operation of the intravenous pump and all the nuances of mixing and infusing the drug.

Miscellaneous

Medicolegal Pitfalls

  • The medicolegal pitfalls involve failure to make the proper diagnosis. Because many of the symptoms of idiopathic pulmonary artery hypertension (IPAH) are nonspecific and because IPAH 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. As above, early therapy may be beneficial in a subset of children.

Special Concerns

  • Counsel patients with moderate-to-severe pulmonary hypertension in the methods of birth control. Labor and delivery is life threatening in patients with significant pulmonary hypertension; therefore, pregnancy should be avoided.
 


More on Pulmonary Hypertension, Idiopathic

Overview: Pulmonary Hypertension, Idiopathic
Differential Diagnoses & Workup: Pulmonary Hypertension, Idiopathic
Treatment & Medication: Pulmonary Hypertension, Idiopathic
Follow-up: Pulmonary Hypertension, Idiopathic
References
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References

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Further Reading

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Keywords

idiopathic pulmonary hypertension, idiopathic pulmonary artery hypertension, IPAH, elevation of pulmonary artery pressure, primary pulmonary hypertension, collagen vascular disease, congenital heart disease, portal hypertension, HIV, Gaucher disease, hereditary hemorrhagic telangiectasia, veno-occlusive disease, chronic obstructive lung disease, interstitial lung disease, sleep-disordered breathing, alveolar hypoventilation, neonatal lung disease, alveolar-capillary dysplasia, pulmonary embolism, right ventricular failure, pulmonary vasoconstriction, patent foramen ovale, tricuspid regurgitation

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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.

Medical Editor

Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis 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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Heidi Connolly, MD, Associate Professor of Pediatrics and Psychiatry, University of Rochester; 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.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
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: Merck Honoraria Speaking and teaching

 
 
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