Thread by @HFpEF

#HFpEF pearl of the day: Eval of filling pressures is critical for HFpEF management. Use entire echo and integrate info for filling pressures assessment: E, E/A, decel time, pulm vein flow, LA volume, LA strain, PASP, PR end-diastolic gradient, IVC, hepatic vein, tricuspid E/e'.

⬆️E, ⬇️DT, ⬆️E/A, and D-dominant pulm vein flow (in older pts); ⬆️E/e’, ⬆️LA volume (esp. ⬆️LA min volume), ⬇️LA reservoir strain; ⬆️PASP, ⬆️PREDP gradient (in absence of PAH); ⬆️tricuspid E/e’ > 6, ⬆️size ⬇️collapsibility of IVC, hep. vein flow reversal all = ⬆️filling pressures

Alternatively, continuous flow by 2D doppler into RA (in RV inflow views) or continuous forward flow on hepatic vein tracing can be helpful signs of normal RA pressure.

Case: 57 yo man with a hx of non-Hodgkin lymphoma, s/p chest XRT 25 yrs ago, mesothelial cell cancer of parotid requiring extensive head/neck resection, remote DVT presents with SOB, chest pain. Coronary CTA: minimal CAD but ++dilated PA, reported as "evidence of PH" on CT report

Came to see me for possible PH due to HFpEF (LVEF was normal at outside hospital) or PAH. Echo showed diastolic septal flattening:

PSAX view showed brisk caval flow into RA:

CMR confirmed diastolic septal flattening. Main PA dilated at 4.4 cm. +LGE of RV at septal insertion points. Invasive hemodynamics: RA 2, RV 27/2, PA 27/6 (mean 14), PCWP 6. What's the diagnosis?

Also, interatrial septum bowing left to right or right to left very helpful for diagnosing imbalance in elevation of LA and RA pressures, respectively, and helpful for diagnosing underlying pathology.

More info: cardiac index 5.6 L/min/m2. No exercise was done during RHC. More info on echo: RA and RV dilated. LA normal size. E/A ratio = 0.6. E/e' < 8 (normal LV filling pressures).

Answer: partial anomalous pulmonary vein return (PAPVR). Right upper pulmonary vein (RUPV) ➡️ SVC. Tip-offs were dilated RA/RV with evidence of RV volume overload (diastolic septal flattening) but normal RA pressure and no evidence of left heart overload. Brisk caval flow into RA

Initial echo c/w over-circulating only the right heart, with no evidence of left heart overload. AV fistula would have overloaded both L+R heart. ASD typically = biatrial enlargement and also overloads L heart. VSDs found in adults mostly bypass the R heart and don't involve RA.

Diastolic septal flattening = RV volume overload. DDx = right heart failure with ⬆️RA pressure, typically in the setting of PAH (due to LV underfilling), severe TR, severe PR, PAPVR. I also often see it in large acute PE due to underfilling of the left heart.

Understanding signs of ⬆️ vs. normal LV and RV filling pressures on echo helped in this case. When I saw the echo, I was struck by brisk caval flow ➡️ RA + diastolic septal flattening + dilated RA/RV and normal left heart. PAPVR seemed most likely, and confirmed on cardiac CT.

This pt underwent surgical correction with a baffle to connect the R upper pulmonary vein ➡️ LA via an ASD created at time of surgery. Chest pain (possibly due to coronary compression from dilated PA) and shortness of breath resolved and he continues to do well ~8 years later.