Left ventricular non-compaction associated with bicuspid aortic...
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Arch Cardiol Mex. 2018;88(5):507---508 www.elsevier.com.mx IMAGE IN CARDIOLOGY Left ventricular non-compaction associated with bicuspid aortic valve and aortic coarctation No compactación del ventrículo izquierdo asociada con válvula aórtica bicúspide y coartación de aorta Nestor A. Parra-Ordo˜ nez, Nydia Avila-Vanzzini, Nilda Espinola-Zavaleta ∗ Laboratory of Echocardiography, National Institute of Cardiology Ignacio Chavez, Mexico City, Mexico Received 29 November 2016; accepted 30 March 2017 Male 18 years old, who began with data of heart failure. The transthoracic echocardiogram showed severe tricus- pid regurgitation and pulmonary hypertension with sPAP of 90 mmHg, bicuspid aortic valve (BAV) with moderate aortic regurgitation, and juxtaductal aortic coarctation. On physical examination, a cardiac apical impulse was palpated, near the midclavicular line in the sixth left inter- costal space. I sound was duplicated by click aortic opening, II sound with physiological splitting and reinforced, aortic systolo-diastolic murmur and a mitral rumble was heard. The peripheral pulses were googly. An interventional treatment of aortic coarctation was performed with Palmaz stent and the patient was kept on medical treatment. In the follow-up, the last echocardiographic study showed severe aortic regurgitation and left ventricular hypertrabeculations with noncompacted/compacted ratio ∗ Corresponding author at: Laboratory of Echocardiography, National Institute of Cardiology Ignacio Chávez, Juan Badiano No. 1, Colonia Sección XVI, Tlalpan, Mexico City, Mexico. E-mail address: [email protected] (N. Espinola-Zavaleta). in short axis at the level of papillary muscle of 3.1, not commented previously, left ventricular dilatation and sys- tolic dysfunction with left ventricular ejection fraction of 40%, moderate tricuspid regurgitation and moderate pul- monary hypertension with sPAP of 64 mmHg and residual coartaction gradient of 40 mmHg (Fig. 1). The patient is waiting for aortic valve replacement, but left ventricu- lar non-compaction (LVNC) with left ventricular systolic dysfunction and pulmonary hypertension will modify his surgical prognosis. Noncompacted cardiomyopathy is characterized by the absence of ventricular compaction and the persistence of deep intertrabecular recesses. The ventricular myocardium is compacted at 5---8 weeks of gestation, progressing from the epicardium to the endocardium and from the base to the apex, reducing the intertrabecular spaces to conform capillaries. The echocardiographic diagnosis is made based on the criteria of Jenni et al. previously described. An end-systolic ratio of non-compacted/compacted layers should be greater than 2. 1 The differential diagnosis should be made with apical hypertrophic cardiomyopathy, dilated cardiomyopathy and with the presence of apical thrombi, diagnoses that are https://doi.org/10.1016/j.acmx.2017.03.007 1405-9940/© 2017 Instituto Nacional de Cardiolog´ ıa Ignacio Ch´ avez. Published by Masson Doyma M´ exico S.A. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Transcript of Left ventricular non-compaction associated with bicuspid aortic...
Arch Cardiol Mex. 2018;88(5):507---508
www.elsevier.com.mx
IMAGE IN CARDIOLOGY
Left ventricular non-compaction associated withbicuspid aortic valve and aortic coarctation
No compactación del ventrículo izquierdo asociada con válvula aórticabicúspide y coartación de aorta
Nestor A. Parra-Ordonez, Nydia Avila-Vanzzini, Nilda Espinola-Zavaleta ∗
Laboratory of Echocardiography, National Institute of Cardiology Ignacio Chavez, Mexico City, Mexico
Received 29 November 2016; accepted 30 March 2017
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adittcoeshould be greater than 2.1
Male 18 years old, who began with data of heart failure.The transthoracic echocardiogram showed severe tricus-pid regurgitation and pulmonary hypertension with sPAP of90 mmHg, bicuspid aortic valve (BAV) with moderate aorticregurgitation, and juxtaductal aortic coarctation.
On physical examination, a cardiac apical impulse waspalpated, near the midclavicular line in the sixth left inter-costal space. I sound was duplicated by click aortic opening,II sound with physiological splitting and reinforced, aorticsystolo-diastolic murmur and a mitral rumble was heard. Theperipheral pulses were googly. An interventional treatmentof aortic coarctation was performed with Palmaz stent andthe patient was kept on medical treatment.
In the follow-up, the last echocardiographic studyshowed severe aortic regurgitation and left ventricularhypertrabeculations with noncompacted/compacted ratio
∗ Corresponding author at: Laboratory of Echocardiography,National Institute of Cardiology Ignacio Chávez, Juan Badiano No.
1, Colonia Sección XVI, Tlalpan, Mexico City, Mexico.E-mail address: [email protected](N. Espinola-Zavaleta).
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https://doi.org/10.1016/j.acmx.2017.03.0071405-9940/© 2017 Instituto Nacional de Cardiologıa Ignacio Chavez. Pubunder the CC BY-NC-ND license (http://creativecommons.org/licenses/b
n short axis at the level of papillary muscle of 3.1, notommented previously, left ventricular dilatation and sys-olic dysfunction with left ventricular ejection fraction of0%, moderate tricuspid regurgitation and moderate pul-onary hypertension with sPAP of 64 mmHg and residual
oartaction gradient of 40 mmHg (Fig. 1). The patient isaiting for aortic valve replacement, but left ventricu-
ar non-compaction (LVNC) with left ventricular systolicysfunction and pulmonary hypertension will modify hisurgical prognosis.
Noncompacted cardiomyopathy is characterized by thebsence of ventricular compaction and the persistence ofeep intertrabecular recesses. The ventricular myocardiums compacted at 5---8 weeks of gestation, progressing fromhe epicardium to the endocardium and from the base tohe apex, reducing the intertrabecular spaces to conformapillaries. The echocardiographic diagnosis is made basedn the criteria of Jenni et al. previously described. Annd-systolic ratio of non-compacted/compacted layers
The differential diagnosis should be made with apicalypertrophic cardiomyopathy, dilated cardiomyopathy andith the presence of apical thrombi, diagnoses that are
lished by Masson Doyma Mexico S.A. This is an open access articley-nc-nd/4.0/).
508 N.A. Parra-Ordonez et al.
A
F G HI
B C D E
RV
RA LV
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Figure 1 Transthoracic bidimensional, continuous and color Doppler and three-dimensional transesophageal echocardiographyshowing bicuspid aortic valve in diastole (A) and systole (B), aortic regurgitation (C), residual aortic coarctation (D and E), ration ), let on (I)
dla
E
Pda
Cd
Rd
R
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on-compacted (blue line)/compacted (red line) layers of 3.1 (Fricuspid regurgitation (H) and moderate pulmonary hypertensi
iscarded using intravenously contrast Definity. In theiterature a concomitant BAV and LVNC was found in 11% of
BAV population.2
thical disclosures
rotection of human and animal subjects. The authorseclare that no experiments were performed on humans or
nimals for this study.onfidentiality of data. The authors declare that no patientata appear in this article.
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ft ventricular spongy aspect in transgastric view (G), moderate.
ight to privacy and informed consent. The authorseclare that no patient data appear in this article.
eferences
. Jenni R, Oechslin E, Schneider J, et al. Echocardiographicand pathoanatomical characteristics of isolated left ventricu-lar non-compaction: a step towards classification as a distinct
cardiomyopathy. Heart. 2001;86:666---71.. Agarwal A, Khandheria BK, Paterick TE, et al. Left ventricularnoncompaction in patients with bicuspid aortic valve. J Am SocEchocardiogr. 2013;26:1306---13.
