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Number 10-09: Acute Pulmonary Edema in an Active Duty Officer in Iraq
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Number 10-09: Acute Pulmonary Edema in an Active Duty Officer in Iraq

Case from: Jonathan Wood, Gerald York, Kevin Steel
Brooke Army Medical Center, Fort Sam Houston, TX 78234 USA

Clinical history: A 27-year-old female active duty soldier with no significant past medical history was evacuated from Iraq for flash pulmonary edema.  Initial work-up demonstrated negative cardiac enzymes at Iraq base as well as on followup at base in Germany. She was transferred to Fort Sam Houston where further workup showed decreased ejection fraction of 25% by 2D echocardiography (Movie 1). A probable diagnosis of left ventricular non-compaction (LVNC) was made however the LV apex was not well visualized on the echocardiogram. She was referred for cardiac MRI for confirmation of the LVNC and for rule out of other etiologies of insidious cardiomyopathy and pulmonary edema.

 

 


Movie 1: 2D Echocardigram

 

Cardiac MRI: Cine images showed left ventricle with global hypokinesis and severe left ventricular systolic function. The LVEF by CMR was 21%. There were significant amount of trabeculae in the LV apex and apico-lateral wall (Movie 2 and 3). Figure 1 demonstrates the maximum thickness of compact myocardium (C) at 8.17 mm and non-compact myocardium (NC) at 21.03 with an NC/C ratio of 2.57.  LGE imaging did not demonstrate contrast enhancement of the myocardium therefore myocardial infarct, active inflammation or infiltration was ruled out. Findings of hypertrabeculation, thinned myocardium, and decreased wall motion with decreased ejection fraction are consistent with left ventricular non-compaction. An NC/C ratio > 2.3 in diastole is highly suggestive of non-compaction by CMR criteria.

 

 

 

Movie 2                                                         Movie 3

 

 

Figure 1

 

Discussion: Left ventricular non-compaction, also commonly known as left ventricular hypertrabeculation, is a rare form of cardiomyopathy caused by intrauterine arrest of cardiac development.  The prevalence has been estimated to be 0.05% in the general population [1].  In utero the fetal heart is an interwoven mesh of myocyte fibers which gradually condenses and compacts.  The compaction starts from the base of the heart and proceeds to the apex with the left ventricle usually becoming more compact than the right.  However an arrest of this compaction in the left ventricle leads to decreased amount of compacted myocardium and hypertrabeculation.

LVNC is rarely diagnosed before the onset of symptoms which are most commonly dyspnea, chest pain, and palpitations. Until recently, the preferred method of diagnosis was echocardiography which showed the NC/C ratio to be greater than 2 in systole.  However, echocardiography cannot reliably evaluate the cardiac apex which is the location of the greatest degree of non-compaction.  CMR is more reliable than echocardiography and is now the preferred diagnostic modality to establish the diagnosis of LVNC.  Petersen et al. have demonstrated that a ratio of NC/C myocardium >2.3 in diastole can reliably establish the diagnosis of LVNC [1,2].  Our patient had an NC/C ratio of 2.57(see figure).  This cutoff is significant as up to 70% of autopsied healthy hearts have shown various degrees of non-compaction [3]. It is important to recognize that hypertrabeculations can be found in various cardiomyopathies and even in some normal left ventricles, therefore close attention should be paid to the identification of thinned compact myocardium coexisting with hypertrabeculation.

LVNC is almost always associated with other congenital malformations with the most common being anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA) and various forms of either left or right ventricular obstruction. [4, 5]. LVNC is also associated with facial dysmorphism, psychomotor retardation, and neuromuscular disorders such as muscular dystrophy, myotonic dystrophy, metabolic myopathies, and Leber's hereditary optic neuropathy [4].

LVNC has a poor prognosis with the most common complications being systolic and diastolic heart failure, thromboembolic events from endocardial thrombus formed in the trabecular recesses and decreased contractility and ventricular arrhythmias.  Treatment of LVNC focuses on these complications with placement of an AICD due to the increased risk of ventricular tachycardia, systemic anticoagulation to prevent thromboembolism, and medical management of heart failure [6].

Perspective:  This case demonstrates:

  • LVNC is a rare form of cardiomyopathy preferentially diagnosed with clinical history and CMR. 
  • LVNC is most accurately diagnosed with an NC/C ratio > 2.3 in diastole at the cardiac apex when using CMR. 
  • The most common sequelae of LVNC are heart failure, thromboembolism, and arrhythmia. 
  • LVNC is associated with neuromuscular disorders and other cardiac malformations.

References:

  1. Petersen SE, Selvanayagam JB, Wiesmann F et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2005 Jul 5;46(1):101-5.
  2. Dodd JD, Holmvang G, Hoffmann U et al. Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. AJR Am J Roentgenol. 2007 Oct;189(4):974-80.

  3. Boyd MT, Seward JB, Tajik AJ et al. Frequency and location of prominent left ventricular trabeculations at autopsy in 474 normal human hearts: implications for evaluation of mural thrombi by two-dimensional echocardiography. J Am Coll Cardiol. 1987 Feb;9(2):323-6.

  4. Chin TK, Perloff JK, Williams RG et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990 Aug;82(2):507-13.

  5. Stöllberger C, Blazek G, Finsterer J. Left ventricular hypertrabeculation/noncompaction requires comprehensive cardiological and neurological examinations. Hum Pathol. 2006 Jan;37(1):122; author reply 123.

  6. Oechslin EN, Attenhofer Jost CH, Rojas JR et al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 2000 Aug;36(2):493-500.
  7. See also the recorded talk LVNC - Myocardial Development and Non-Compaction by Nigel Brown, at the Cardiomyopathy Association 2009 meeting in London, UK.

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Number 10-09 Acute Pulmonary oedema...
Hi, this 2010 case of LVNC - perhaps we would not always state that LVNC is almost always associated with congenital abnormalities, and perhaps the discussion here is over-simplistic for our current understanding.See this scmr talk
On: 10/11/2011 By: moon Read more?

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