What hemodynamic parameters are consistent with a diagnosis of idiopathic pulmonary arterial hypertension (IPAH) in a patient undergoing right heart catheterization?

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Differential Diagnosis for Worsening Dyspnea on Exertion

Single Most Likely Diagnosis

  • Idiopathic pulmonary arterial hypertension (IPAH): This diagnosis is consistent with the patient's symptoms, echocardiogram results (right ventricular hypertrophy), and the planned right heart catheterization. The correct answer choice that supports this diagnosis is A. Pulmonary capillary wedge pressure of ≤15 and absence of pulmonary veno-occlusive disease, as IPAH is characterized by elevated pulmonary arterial pressure with a normal pulmonary capillary wedge pressure.

Other Likely Diagnoses

  • Obesity-related respiratory disease: The patient's obesity (BMI of 35) may contribute to her dyspnea, and further evaluation is needed to determine the extent of its impact.
  • Sleep apnea: Although not directly related to the provided answer choices, the patient's obesity and symptoms suggest the possibility of sleep apnea, which could be contributing to her dyspnea.
  • Cardiomyopathy: Although the left ventricular ejection fraction is 60%, which is within the normal range, further evaluation is needed to rule out cardiomyopathy, particularly given the patient's obesity and symptoms.

Do Not Miss Diagnoses

  • Pulmonary veno-occlusive disease (PVOD): This condition can present similarly to IPAH but requires different management. It is essential to rule out PVOD, as the treatment approach differs significantly.
  • Left heart disease: Although the pulmonary capillary wedge pressure is expected to be ≤15 in IPAH, it is crucial to rule out left heart disease, which could cause elevated pulmonary capillary wedge pressure and contribute to the patient's symptoms.
  • Chronic thromboembolic pulmonary hypertension (CTEPH): This condition can cause similar symptoms and requires different management. It is essential to consider CTEPH in the differential diagnosis.

Rare Diagnoses

  • Other rare causes of pulmonary hypertension, such as:
    • Connective tissue disease-associated pulmonary arterial hypertension
    • Congenital heart disease-associated pulmonary arterial hypertension
    • HIV-associated pulmonary arterial hypertension
    • Other rare conditions that can cause pulmonary hypertension, such as pulmonary capillary hemidesmosis or unclassified forms of pulmonary vascular disease.

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This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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