Case Presentation:
A 46-year-old female with past medical history of Group 1 pulmonary hypertension (last known PASP 114 mm Hg from 2 months prior), on triple therapy for PAH (sGC, ERA, and prostinoids); cor pulmonale, and cirrhosis. She presented with worsening shortness of breath (SOB) and anasarca, along with vital signs indicative of cardiogenic shock.
Upon arrival, the patient had a pulse of 94 beats/minute and blood pressure of 94/62 mm Hg, requiring 4 L of supplemental oxygen via nasal cannula. She was admitted to the intensive care unit (ICU) for inhaled veletri administration and initiation of intravenous vasopressors.
Physical examination revealed prominent jugular venous distention (JVD) and marked anasarca, with 3+ pitting edema throughout the abdomen, bilateral lower extremities, and 2+ edema in the upper extremities and thorax.
A chest X-ray performed in the emergency department showed massively enlarged cardiac silhouette, suggestive of severe pericardial effusion.
POCUS Findings:
1. Parasternal Long-Axis View (PLAX):
In the PLAX view, which typically does not display the right atrium (RA), a massively dilated RA was visible. The dilation of the RA was attributed to chronic pulmonary hypertension.
Although PLAX can be used to comment on the structure of RVOT, in this view enlarged RA distorts the standard plane of PLAX used for this assessment.
Moreover, as a thumb rule, RVOT, LA, and aortic outflow tract appear more or less similar in size. Here, if we compare RVOT with left ventricular outflow- RVOT is significantly larger.
2. Parasternal Short-Axis View (PSAX):
The PSAX view revealed a dilated RV with a prominent "D sign" observed during diastole, indicating volume overload.
During systole, the D sign was comparatively less pronounced, suggesting a combination of volume and pressure overload.
The PSAX view also revealed the presence of circumferential pericardial effusion.
3. Apical View:
The massively enlarged RA is once again evident. Additionally, the left ventricle (LV) appeared hyperdynamic, with the mitral leaflets approximating well towards the LV wall during systole.
Not only does the RA appear larger than LA in this view, RA seems to be THE LARGEST chamber in this view!
Formal echo findings for reference:
The left ventricle is very small and D shaped. The estimated LVEF 60%. Paradoxical septal motion due to RV pressure and volume overload.
Severe tricuspid functional and eccentric regurgitation with estimated PA systolic pressure is 82 mmHg (lower c/t prior echo) . Findings c/w group I PAH.
Massive right atrial enlargement.
Moderate pulmonic regurgitation.
Right ventricular systolic function is reduced with hypoinesis of distal RV wall. Severe right ventricular dilatation.
Large localized pericardial effusion around RA.
Elevated central venous pressure and dilated Hepatic vein
Hospital Course and Management:
Diuresis was initially attempted with bumex bolus and drip; however, an inadequate response necessitated the initiation of continuous renal replacement therapy (CRRT). Pulmonology consultation led to a plan for repeat right heart catheterization (RHC) to assess right-sided pressures and prognosis. The subsequent management focused on achieving euvolemia to ensure accurate right-sided pressure measurements unaffected by hypervolemia. Over a week of ICU stay, a net negative fluid balance of 30 L was achieved, following which the RHC was performed to obtain pressure measurements.
Right heart catheterization findings:
1. PA Pressure: (previously known PA pressure from 2 months prior- PA 114/30; 60 )
2. RA Pressure: (no prior RA pressure on file.)
Other findings:
RV pressure: 98 PA: 98/ 50/ 66 . Cardiac output was 4.12 by Fick. Cardiac index is 2.33 L/min/m2. O2 Sat: PA 48%.
She subsequently responded to bumex drip and CRRT was discontinued. However, over her hospital course, she deteriorated hemodynamically with increasing pressor requirements. After goals of care discussions, family chose comfort care and the patient deceased.
Discussion:
Anasarca only mildly improved with a net negative diuresis of 30 L. This points towards the chronicity of anasarca in this patient. Her pulmonary artery (PA) pressures must have been elevated for a significant amount of time to persistently keep central venous pressure (CVP) high, causing severe interstitial edema in the skin and soft tissues. It is fascinating to imagine that in a steady state, being anasarcic would be their baseline.
Based on the echocardiogram findings, it is clear that severe pulmonary hypertension has caused significant structural changes to the right heart, probably over the course of months to years.
The signs of right ventricular (RV) volume overload observed here do not necessarily specify acuity. D sign is sensitive for RV strain, but not specific for acute vs chronic. In other words, even if this patient was in her baseline state of health when she was at home before getting sick, the D sign can still be seen. In some cases, likely applicable to this patient as well, patients live chronically with RV strain and some degree of septal bowing.
Finally, coming to the most interesting point from the right heart catheterization results, comparing the two results from 2 months apart, the PA pressures changed from 114/30 (60) mm Hg to 99/35 (66) mm Hg.
However, there was a decrease in pulmonary artery systolic pressure (PASP) from 114 to 99 mm Hg. (Also, non-invasive PASP was 82 mm Hg from the echocardiogram done on admission.) Given the clinical deterioration along with this objective data, it emphasizes the worsening of right heart failure. In other words, the RV has become weaker, to the point that it is not able to contract with enough strength to generate a PASP of 114 mm Hg in its outflow. Hence, it was the chronic worsening of the heart to a point of no return, secondary to relatively stable but severe and chronic pulmonary hypertension, which manifested as the critical illness in our patient.
Author:
Neev Patel, MD
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