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Acute Mitral Regurgitation: Another Great Masquerader?

Rinjal Brahmbhatt MD, Jacqueline Kehler MD, and Jonathan Keevil MD

University of Wisconsin School of Medicine and Public Health

Dyspnea and a Murmur

References

Case  87 YOF with a history of HTN and osteoporosis was admitted to the hospital with acute-onset SOB both at rest and with exertion that began the day prior to admission and was associated with chest tightness, fatigue, and orthopnea  No f/c, cough, wheezing, hemoptysis, lower extremity edema, weight gain, or PND  Possible sick contacts at her assisted living facility  Recently traveled to Missouri  No hx of known lung disease, pets, occupational exposures, TB risk factors, hx of VTE, or hx of tobacco use  Home medications: acetominophen prn, calcium, vitamin D, and lisinopril-HCTZ  Vitals: 36.8, 142/65, 121, 23, 96% on CPAP  Exam was remarkable only for a 2/6 holosystolic murmur heard best at the apex with radiation to the axilla.

Significant Labs WBC 15.1 BUN 25 Cr 1.05 Troponin <0.02 BNP 430 D-dimer 2.4 ABG 7.38/39/279/23 on 100% FiO2

ECG Sinus tachycardia, no ischemic changes

Imaging  CXR showed asymmetric opacities, right > left (Figure 1).  Chest CT angiogram showed predominantly right upper lobe abnormalities with ground glass opacities, intralobular septal thickening, and consolidation (Figure 2).

Clinical Course  Despite IV diuresis for suspected decompensated HF and empiric broad-spectrum antibiotics for a possible atypical pneumonia, she did not improve.  Given the new murmur on exam, a TTE was obtained and showed no wall motion abnormalities, hyperdynamic LV with EF 80%, MV prolapse of the anterior leaflet without regurgitation, grade 2 diastolic dysfunction, normal RV size and function, and PASP of 40 mmHg.  A follow-up TEE showed severe MR directed eccentrically (which explained the lack of TTE findings) and posteriorly with severe flail motion of the anterior leaflet due to chord rupture (Figure 3).  As evidenced by Doppler images, the regurgitant jet generated more significant flow reversal in the right pulmonary vein than it did in the left, which could explain the unilateral findings on chest imaging (Figures 4 & 5).  Due to advanced age and comorbidities, our patient chose medical management with afterload reduction rather than surgical valve repair.

Causes of Acute MR  MI  Papillar muscle rupture  Infective endocarditis  Blunt chest trauma  Acute rheumatic fever  Myxomatous degeneration as in Marfan’s  HOCM  Atrial myxoma  Idiopathic chordae tendinaeae rupture due to spontaneous rupture  Sepsis  Intramural amyloidosis  Coronary vasospasm

Figure 2. Chest CTA with predominantly RUL abnormalities: ground glass opacities, intralobular septal thickening, consolidation.

Figure 1. CXR with asymmetric opacities, right > left.

Clinical Presentation of Acute MR

 Sudden, rapid onset of pulmonary edema  Hypotension +/- cardiogenic shock  Acute right-sided heart failure  SOB, DOE, fatigue, weakness  Exam with tachycardia, peripheral vasoconstriction with thready pulses, pallor, diaphoresis, elevated JVD  If TR has developed, there may be a RV lift  Soft, low-pitched systolic murmur along the left sternal border that may radiate to the back  S3, loud P2 may be present

Diagnosis of Acute MR

• Abdullah HN, Khairina WW. Pulmonary edema of an unexpected cause. South Med J 2008 Oct; 101(10):1035-7. • Alcalde O, Domingo E, Figueras J. Recurrent severe acute pulmonary edema caused by transient left ventricular insufficiency with mitral regurgitation related to severe coronary artery spasm. Circ Heart Fail 2010 Mar; 3(2):332-5. • Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr, Faxon DP, Freed MD, Gaasch WH, et al. 2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease. Journal of the American College of Cardiology 2008; 52(13): e1–e142. • Castello R, Fagan L, Lenzen P, Pearson AC, Labovitz AJ. Comparison of transthoracic and transesophageal echocardiography for assessment of left-sided valvular regurgitation. The American Journal of Cardiology 2001; 68(17): 1677-1680. • Cebeci BS, Kardesoglu E, Kilicaslan F, Kirilmaz A, Ulusoy E, Ozmen N, Uz O, Dincturk M. Unilateral pulmonary edema secondary to idiopathic rupture of mitral chordaea tendinae. South Med J 2006; 99(5):549-50. • Chatterjee K, Parmley WW, Swan HJ, Berman G, Forrester J, Marcus HS. Beneficial effects of vasodilator agents in severe mitral regurgitation due to dysfunction of subvalvar apparatus. Circulation 1973; 48(4): 684–690. • D'Aloia A, Faggiano P, Brentana L, Boldini A, Procopio R, Racheli M, Dei Cas L.A difficult diagnosis: right unilateral cardiogenic pulmonary edema. Usefulness of biochemical markers of heart failure for the correct diagnosis. Ital Heart Journal 2005 Sep; 6(9):771-4. • Giuliano JS Jr, Sekar P, Dent CL, Border WL, Hirsch R, Manning PB, Wheeler DS. Unilateral pulmonary edema and acute rheumatic fever. Eur J Pediatr 2008 Apr; 167(4):465-7. • Hutyra M, Skala T, Marek D, Nemec P, Sluka M, Ostransky J, Richter M, Lukl J.Acute severe mitral regurgitation with cardiogenic shock caused by two-step complete anterior papillary muscle rupture during acute myocardial infarction. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2006 Nov; 150(2):293-7. • Salinas P, Martín-Reyes R, Peinado R, Ramírez U. Unilateral pulmonary edema and shock: a diagnostic challenge. Intensive Care Medicine 2009; 35(11): 2000-2001 • Shin JH, Kim SH, Park J, Lim YH, Park HC, Choi SI, Shin J, Kim KS, Kim SG, Hong MK, Lee JU. Unilateral pulmonary edema: a rare initial presentation of cardiogenic shock due to acute myocardial infarction. J Korean Med Sci. 2012 Feb; 27(2):211-4

Figure 3. TEE showing mitral valve with flailed leaflet.

Figure 4. TEE showing Left Upper Pulmonary Vein (LUPV) and minimal systolic flow reversal on Doppler.

Figure 5. TEE showing Right Upper Pulmonary Vein (RUPV) and systolic flow reversal on Doppler.

LUPV

RUPV

Low Flow Reversal

Flow Reversal

Transthoracic Echocardiography (TTE)  Demonstrates valve disruption and provide semi-quantitative measure of regurgitation severity  Limited sensitivity: misses up to 25% of MR seen on TTE  Only finding may be hyperdynamic left ventricular function

Transesophageal Echocardiography (TEE)  Obtain if TTE is not diagnostic and patient has acute heart failure and hyperdynamic left ventricular function on TTE  Provides more detailed information about anatomic etiology  Is favored for surgical planning

Treatment

 Asymmetric or unilateral pulmonary edema is a rare cause of focal abnormalities on chest imaging and is frequently mistaken for more common conditions such as infection or malignancy.

 The asymmetry of pulmonary infiltrates in MR is due to the asymmetrical jet of mitral regurgitation causing a selective increase in R upper pulmonary vein pressure and consequently, a greater degree of R-sided pulmonary edema.

 There are clinically important limitations in relying on TTE to diagnose acute MR. In a patient with symptoms suggestive of acute heart failure and hyperdynamic LV function on TTE, acute MR should be considered and a TEE should be obtained.

 This case highlights the importance of recognizing the variation in clinical manifestations of acute mitral regurgitation.

 Physicians should include acute valvular regurgitation in the differential diagnosis of any patient presenting with pulmonary manifestations, even with focal findings on chest imaging.

Conclusions

 Typically a bridge to emergent surgical repair or replacement

 Nitroprusside to reduce afterload, improve left ventricular forward flow, and reduce pulmonary vascular congestion

 If hypotensive, ionotropic agents and an intra-aortic balloon pump may be required in addition to nitroprusside

Cardiac  Acute MR  Acute MI without MR  L sided HF (rare)

 Pulmonary vein obstruction  ARDS  Alveolar hemorrhage  Lung infarction

Pulmonary  Pneumonia  Aspiration  Acute pneumonitis  Malignancy  Re-expansion pulmonary edema after thoracentesis  Bronchial obstruction

Differential Diagnosis of Unilateral Pulmonary Infiltrates