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Exertional dyspnoea in a 28-year-old woman
  1. Selma D Carlson,
  2. Zachary L Steinberg,
  3. Eric V Krieger
  1. Department of Medicine, Division of Cardiology, University of Washington Medical Center, Seattle, Washington, USA
  1. Correspondence to Dr Selma D Carlson, Department of Medicine, Division of Cardiology, University of Washington Medical Center, Seattle, Washington, USA; selmad{at}uw.edu

Abstract

Clinical introduction A 28-year-old woman with a history of critical pulmonic stenosis, status postsurgical valvotomy and subsequent pulmonary valve replacement, presented to the cardiology clinic with 1 year of progressive exertional dyspnoea. She has a heart rate of 75 bpm and blood pressure of 110/55 mm Hg. Cardiac auscultation reveals a 1/6 systolic ejection murmur along the left sternum and an early 3/6 diastolic decrescendo murmur. A transthoracic echocardiogram is obtained (figure 1).

Questions Which of the following would be most likely found during right heart catheterisation?

  1. Ratio of pulmonary to systemic blood flow (Qp:Qs) >1.5

  2. Pulmonary vascular resistance >3 Wood units

  3. Right atrial pressure >10mm Hg

  4. Pulmonary artery systolic pressure >45mm Hg E. Pulmonary artery diastolic pressure <10mm Hg

Questions

  • Pulmonic Valve Disease
  • Congenital Heart Disease
  • Echocardiography
  • Valve Disease Surgery

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Answer: C. Right atrial pressure >10 mm Hg

Figure 1

(A) Pulsed-wave Doppler tracing of transpulmonic flow. (B) Continuous-wave Doppler tracing of transtricuspid flow.

Figure 1A demonstrates significant pulmonary regurgitation into a restrictive right ventricle (RV). During early diastole, regurgitation from the pulmonary artery (PA) into the RV is present. In late diastole, antegrade flow from the RV into the PA is present, a finding seen in restrictive RV filling. In the setting of restrictive physiology, atrial contraction in late diastole forces blood into a poorly compliant RV resulting in premature opening of the pulmonic valve with antegrade flow on spectral and colour Doppler.1 2 Restrictive RV physiology is common in patients with congenital RV outflow tract obstruction, seen in conditions such as Tetralogy of Fallot and pulmonary atresia, sometimes even after timely repair.3 Delayed repair of the obstruction may result in myocardial fibrosis and restrictive physiology, which is associated with poor clinical outcomes in some, but not all, studies.4 High right atrial pressures would be expected in the presence of a non-compliant RV, therefore, answer C is correct. There is no flow from the LVOT to RVOT in figure 1A to suggest a supracristal VSD, so answer A is incorrect. Figure 1B shows the transtricuspid pressure gradient to be 19 mm Hg, inconsistent with pulmonary hypertension, so B and D are incorrect. Near equalisation of diastolic PA pressures and RV end-diastolic pressures is expected in the setting of severe pulmonary regurgitation. Since RV end-diastolic pressures are likely to be high given RV restriction, answer E is incorrect.

References

Footnotes

  • Contributors All authors participated in the writing and editing of the manuscript, as well as data collection.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.