Services & Specialties
Pulmonary Hypertension

Comprehensive Diagnostic and Long-Term Treatment for Patients with Pulmonary Hypertension

Part of Cincinnati Children's Heart Institute, the Pulmonary Hypertension Clinic provides comprehensive diagnostic and long-term treatment and follow-up for patients with pulmonary hypertension. In addition, our clinic has particular interest in infants with congenital diaphragmatic hernias (CDH) with resulting pulmonary hypertension. We provide both inpatient and outpatient consultation and follow-up care.

About Pulmonary Hypertension

Pulmonary hypertension results from increased resistance of blood flow through the pulmonary vasculature (due to progressive muscular thickening and fibrosis of the blood vessels in the lungs). Pulmonary hypertension is a chronic and progressive disease that often requires long term care with our team.

Commonly mistaken for asthma, this disease forces the heart’s right ventricle to work harder, causing it to enlarge, thicken and experience an elevation in pressure. In the early stages, the changes in the blood vessels may be reversible, but do become permanent later. If this pressure is not relieved, right ventricular failure, arrhythmias and even death may result.

Read more about Pulmonary Hypertension in the Heart Encyclopedia.

Our Expertise

The Pulmonary Hypertension Program provides comprehensive diagnostic and long-term treatment and follow-up for patients with pulmonary hypertension. Our comprehensive multi-disciplinary team comprises dedicated physicians, nursing staff, pharmacists, dietitians, social workers and genetic counselors. We work closely with other programs at Cincinnati Children’s in an integrated manner to bring the best care possible to our patients whose disease process may be complicated by pulmonary hypertension. These include newborn medicine, pulmonary, liver service and Hemangioma / Vascular Malformation teams, among others.

The Pulmonary Hypertension Program also works alongside The Heart Institute Research Core (HIRC) to participate in pediatric specific pulmonary hypertension cutting edge research. HIRC is a full-service, academic, clinical and translational research organization within the Heart Institute at Cincinnati Children’s Hospital Medical Center (CCHMC). HIRC enables and accelerates Clinical and translational science by facilitating the design, implementation, dissemination, and application of innovative cardiovascular research studies.

Pulmonary Hypertension Comprehensive Care Center

We are proud to be one of eight pediatric centers in the nation to earn accreditation from the Pulmonary Hypertension Association (PHA) as a Pulmonary Hypertension Accredited Comprehensive Care Center.

PHA established the Pulmonary Hypertension Care Center (PHCC) Program to identify centers with expertise in pulmonary hypertension that have demonstrated an ability to properly diagnose the disease and have the capability to manage these complex patients. The PHA PHCC Accreditation Program is a critical landmark, as patients now have a means to find high-quality pulmonary hypertension programs throughout the U.S.

As a PHCC, we participate in the PHA Registry (PHAR), a multi-center, prospective, observational registry of newly evaluated patients diagnosed at accredited U.S. centers with either pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH).

Pulmonary Hypertension FAQ's

The long-term prognosis depends on its cause, the severity of the disease at the time of diagnosis and response to therapy. Recent advances in medical therapies have had a profound impact on the outcome of pulmonary hypertension.
Pulmonary hypertension may be idiopathic, with no identifiable cause, or inherited. However, secondary pulmonary hypertension may occur in many types of diseases including congenital heart disease, lung disease, various connective tissue diseases and sickle cell disease.
In the early stages, pulmonary hypertension may be asymptomatic. However, as the disease progresses, non-specific symptoms, such as increased fatigue and occasional shortness of breath, may become apparent. With further deterioration, increasing dyspnea with less exertion, dizziness, palpitations and syncope may be present. Chest pain and peripheral edema may be present when pulmonary hypertension is severe. Unfortunately, many of the symptoms may mimic other pulmonary syndromes (such as asthma), thus necessitating a higher level of suspicion for this disease.
Early clinical presentation signs may be subtle, and may include only a prominent second heart sound. With progression, more obvious signs related to elevated right ventricular and right atrial pressure may present, such as jugular vein distention, palpable second heart sound, hepatomegaly and peripheral edema. Murmurs may or may not be present.
A good history and physical exam can give you clues to diagnosis along with additional testing, including electrocardiogram, chest X-ray, echocardiogram, six-minute walk test, MRI scanning and the gold standard for diagnosis, cardiac catheterization.
While there is no cure for pulmonary hypertension, there are many treatments that have been found to relieve some of the symptoms and slow the progression of the disease. These include oxygen, diuretics, calcium channel blockers, phosphodiesterase-5 (PDE5) inhibitors, endothelin receptor antagonists (ERAs), anticoagulant therapies and prostanoids, including prostacyclin. In addition, your team may refer you to cardiac rehabilitation to maximize your health and fitness goals. If your PH progresses, you made be referred for lung transplantation or Pott’s Shunt.

Blog: How We've Adapted to Our Daughter's Condition

Pulmonary Hypertension: Adapting to Daughter's Condition.
Kelly, Riley's mom, feels fortunate that her daughter avoided the serious symptoms of pulmonary hypertension while they sought answers for Riley’s shortness of breath. Three years post-diagnosis, Riley is back to normal life with a few modifications, including a "dry" suit! Read Riley's Story

Hank The Heart Presents: What is Pulmonary Hypertension?

PH Care Centers.

The Pulmonary Hypertension Clinic at Cincinnati Children’s is a Pulmonary Hypertension Association (PHA) Accredited Pediatric Center of Comprehensive Care.