Exercise Pulmonary Hypertension

A 74-year-old male patient with a history of chronic kidney disease stage III and paroxysmal atrial fibrillation was referred to cardiology with profound shortness of breath with exertion. He was previously quite active and hiked frequently in the deserts of Arizona. Over the last 6 months, he noted inability to complete his usual hiking routes and labored breathing with simple activities such as showering and household chores. He had normal vital signs, and his exam was remarkable for a well-built male in no acute distress. His lungs were clear to auscultation. His heart rate was regular with a split S2 with inspiration, no S3/S4, and no murmurs, rubs, clicks, gallops, or edema. His neck veins were elevated to 9 cm H20, and the hepatojugular reflex was negative. Pulmonary function testing was unremarkable for his age, and an electrocardiogram demonstrated sinus rhythm with first degree atrioventricular block and right axis deviation.

He underwent stress echocardiogram. At rest, his left ventricular ejection fraction was 55%, which increased to 70% at peak exertion. Echocardiographic evaluation of the right heart noted resting right ventricular systolic pressure of 50 mmHg, tricuspid annular plane systolic excursion of 15 mm, and fractional area change of 35%. Stress images captured a peak tricuspid regurgitation jet of 3.75 m/s. No wall motion abnormalities or septal bowing were seen. He was referred for right heart catheterization with invasive cardiopulmonary exercise testing. Pulmonary artery and right atrial wave forms and hemodynamic data are shown in Figure 1 and Table 1.

Figure 1: Telemetry, Pulmonary Artery, and Right Atrial Wave Forms at 60 Watts Workload

Figure 1
Scale set to 100 mmHg.

Table 1: Right Heart Catherization Data at Rest and at Peak Exertion

Resting Hemodynamics

Hemodynamics at 60 Watts Workload

Systemic Blood Pressure: 112/76 (86) mmHg

Systemic Blood Pressure: 132/82 (98) mmHg

Heart Rate: 65 bpm

Heart Rate: 123 bpm

Right Atrial Pressure: 6/5 (4) mmHg

Right Atrial Pressure: 17/14 (14) mmHg

Right Ventricular Pressure: 29/2 (13) mmHg

Right Ventricular Pressure: Not available

Pulmonary Artery Pressure: 30/12 (16) mmHg

Pulmonary Artery Pressure: 88/28 (52) mmHg

Pulmonary Capillary Occlusion Pressure: (10) mmHg

Pulmonary Capillary Occlusion Pressure: (11) mmHg

Cardiac Output
-Thermodilution: 4.68 L/min
-Fick: 3.89 L/min

Cardiac Output
-Fick based on invasive peak V02: 5.79 L/min

Pulmonary Vascular Resistance: 3.3 Woods units

Pulmonary Vascular Resistance: 7.8 Woods units

Transpulmonary Gradient: 13 mmHg
Diastolic Pressure Gradient: 3 mmHg

Transpulmonary Gradient: 41 mmHg
Diastolic Pressure Gradient: 3 mmHg

Aortic Saturation: 98%
Pulmonary Artery Saturation: 70%

Aortic Saturation: 89%
Pulmonary Artery Saturation: 29%

Values reported as systolic/diastolic and (mean pressure).

Given the patient's hemodynamic data, which of the following medications is indicated for therapy?

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