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Isolated noncompacted myocardium in an elderly patient.

Miranda EP, Albuquerque LA, Menezes RA, Silva AW, Belém Lde S, Ramos FP - Clinics (Sao Paulo) (2008)

View Article: PubMed Central - PubMed

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Noncompaction of the myocardium (NCM) is a rare disorder that is characterized by prominent myocardial trabecularizations and deep intertrabecular recesses, leading to a spongy appearance of the myocardium... During the fifth to the eighth week of development, these recesses are supposed to turn into capillaries as the ventricular myocardium gradually compacts., Although the exact mechanisms that generate NCM remain unclear, it has been suggested that an arrest of cardiac development during the compaction of myocardial fibers may lead to the persistence of multiple trabeculations within the ventricular myocardium., A 76-year-old woman with a 5-month history of progressive dyspnea presented with significant worsening one week prior to admission... Three-dimensional transthoracic echocardiograpy (TTE) demonstrated the following: moderate enlargement of the left ventricle (LV), severe enlargement of the left atrium (LA) at 5.7 cm and a large right atrium (RA)... In addition, the patient was found to have severe mitral regurgitation (Figure 3), moderate tricuspid regurgitation, pulmonary hypertension (systolic pressure of the pulmonary artery = 52 mmHg), and patent foramen ovale with minimal right-to-left shunting... After nine days, the patient was discharged with mild limitation of activity (New York Heart Association II), and prescribed the following medications: an angiotensin-conversing enzyme inhibitor, an aldosterone antagonist, a beta-adrenergic blocker, and oral anticoagulant therapy... NCM is a rare entity, usually diagnosed in the pediatric population and is associated with other structural congenital malformations of the heart, such as obstruction of the right or left ventricle outflow tract... It is believed to be genetically transmitted, and there is a male predominance ranging from 56% to 82% among the reported familial cases., Isolated noncompaction of the myocardium (INCM), defined by the absence of any other structural cardiac abnormalities, is an even rarer form of the entity, with only a few reports in the literature. – Our patient is a rather unusual case of INCM, since her diagnosis was established at age 76... This is significantly outside of the established age range of adults with this disorder, which is 37 to 43 years old., In the literature, we identified only seven reported cases of INCM described in patients older than 70 years, and this case represents one of the oldest patients ever reported... There are several explanations for this late diagnosis: 1) symptoms may manifest late since there is significant variability in the natural course of INCM; 2) This patient was very sedentary, perhaps rendering the symptoms less prominent; 3) The patient lived in a rural area that had restricted access to the healthcare system, so she may have suffered from mild symptoms of heart failure that went unnoticed for a significant amount of time... The patient met echocardiographic criteria for the diagnosis of INCM:,,, 1) The presence of multiple echocardiographic trabeculations, particularly in the apex and free wall of the LV; 2) Multiple deep intratrabecular recesses communicating with the ventricle cavity, as demonstrated by color Doppler imaging; 3) a 2-layered structure of the endomyocardium with an increased noncompacted to compacted ratio (>2.0 in adults, >1.4 in children); 4) the absence of another coexisting heart anomaly, including aortic and pulmonary valve abnormalities and coronary disease... The mitral valve affliction is believed to be secondary to the ventricular dysfunction and dilatation caused by INCM... It was treated with beta-blockers and anticoagulant therapy to ensure rhythm control and to prevent clot formation., The increasing number of reported cases of INCM, the high variability of its clinical manifestations, and the diagnosis of INCM in an elderly patient lead us to believe that this disease is actually underestimated... Further research is needed to solve current controversies regarding diagnostic criteria, nomenclature, prognosis, origin and pathogenesis... Some remaining questions include whether or not the presence of congenital heart anomalies should exclude NCM, and whether cases diagnosed in young children or in adults represent subgroups of the disease.

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Transthoracic achocardiography at apical 4-chamber (AFC) view showing patente foramen ovale, presence of numerous prominent trabeculations and deep intertrabecular recesses. AD - right atrium; VC - right ventriculum; AE - left atrium; VE - left ventriculum
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f4-cln63_4p0567: Transthoracic achocardiography at apical 4-chamber (AFC) view showing patente foramen ovale, presence of numerous prominent trabeculations and deep intertrabecular recesses. AD - right atrium; VC - right ventriculum; AE - left atrium; VE - left ventriculum

Mentions: Three-dimensional transthoracic echocardiograpy (TTE) demonstrated the following: moderate enlargement of the left ventricle (LV), severe enlargement of the left atrium (LA) at 5.7 cm and a large right atrium (RA). In addition, the patient was found to have severe mitral regurgitation (Figure 3), moderate tricuspid regurgitation, pulmonary hypertension (systolic pressure of the pulmonary artery = 52 mmHg), and patent foramen ovale with minimal right-to-left shunting. Furthermore, the TTE showed numerous prominent trabeculations, and deep intertrabecular recesses (Figure 4 and 5), along with diffuse and moderate hypokinesia of the LV with LV systolic function mildly impaired (EF = 49%). According to the echocardiographic criteria, isolated noncompacted myocardium (INCM) was diagnosed.


Isolated noncompacted myocardium in an elderly patient.

Miranda EP, Albuquerque LA, Menezes RA, Silva AW, Belém Lde S, Ramos FP - Clinics (Sao Paulo) (2008)

Transthoracic achocardiography at apical 4-chamber (AFC) view showing patente foramen ovale, presence of numerous prominent trabeculations and deep intertrabecular recesses. AD - right atrium; VC - right ventriculum; AE - left atrium; VE - left ventriculum
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2664141&req=5

f4-cln63_4p0567: Transthoracic achocardiography at apical 4-chamber (AFC) view showing patente foramen ovale, presence of numerous prominent trabeculations and deep intertrabecular recesses. AD - right atrium; VC - right ventriculum; AE - left atrium; VE - left ventriculum
Mentions: Three-dimensional transthoracic echocardiograpy (TTE) demonstrated the following: moderate enlargement of the left ventricle (LV), severe enlargement of the left atrium (LA) at 5.7 cm and a large right atrium (RA). In addition, the patient was found to have severe mitral regurgitation (Figure 3), moderate tricuspid regurgitation, pulmonary hypertension (systolic pressure of the pulmonary artery = 52 mmHg), and patent foramen ovale with minimal right-to-left shunting. Furthermore, the TTE showed numerous prominent trabeculations, and deep intertrabecular recesses (Figure 4 and 5), along with diffuse and moderate hypokinesia of the LV with LV systolic function mildly impaired (EF = 49%). According to the echocardiographic criteria, isolated noncompacted myocardium (INCM) was diagnosed.

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Noncompaction of the myocardium (NCM) is a rare disorder that is characterized by prominent myocardial trabecularizations and deep intertrabecular recesses, leading to a spongy appearance of the myocardium... During the fifth to the eighth week of development, these recesses are supposed to turn into capillaries as the ventricular myocardium gradually compacts., Although the exact mechanisms that generate NCM remain unclear, it has been suggested that an arrest of cardiac development during the compaction of myocardial fibers may lead to the persistence of multiple trabeculations within the ventricular myocardium., A 76-year-old woman with a 5-month history of progressive dyspnea presented with significant worsening one week prior to admission... Three-dimensional transthoracic echocardiograpy (TTE) demonstrated the following: moderate enlargement of the left ventricle (LV), severe enlargement of the left atrium (LA) at 5.7 cm and a large right atrium (RA)... In addition, the patient was found to have severe mitral regurgitation (Figure 3), moderate tricuspid regurgitation, pulmonary hypertension (systolic pressure of the pulmonary artery = 52 mmHg), and patent foramen ovale with minimal right-to-left shunting... After nine days, the patient was discharged with mild limitation of activity (New York Heart Association II), and prescribed the following medications: an angiotensin-conversing enzyme inhibitor, an aldosterone antagonist, a beta-adrenergic blocker, and oral anticoagulant therapy... NCM is a rare entity, usually diagnosed in the pediatric population and is associated with other structural congenital malformations of the heart, such as obstruction of the right or left ventricle outflow tract... It is believed to be genetically transmitted, and there is a male predominance ranging from 56% to 82% among the reported familial cases., Isolated noncompaction of the myocardium (INCM), defined by the absence of any other structural cardiac abnormalities, is an even rarer form of the entity, with only a few reports in the literature. – Our patient is a rather unusual case of INCM, since her diagnosis was established at age 76... This is significantly outside of the established age range of adults with this disorder, which is 37 to 43 years old., In the literature, we identified only seven reported cases of INCM described in patients older than 70 years, and this case represents one of the oldest patients ever reported... There are several explanations for this late diagnosis: 1) symptoms may manifest late since there is significant variability in the natural course of INCM; 2) This patient was very sedentary, perhaps rendering the symptoms less prominent; 3) The patient lived in a rural area that had restricted access to the healthcare system, so she may have suffered from mild symptoms of heart failure that went unnoticed for a significant amount of time... The patient met echocardiographic criteria for the diagnosis of INCM:,,, 1) The presence of multiple echocardiographic trabeculations, particularly in the apex and free wall of the LV; 2) Multiple deep intratrabecular recesses communicating with the ventricle cavity, as demonstrated by color Doppler imaging; 3) a 2-layered structure of the endomyocardium with an increased noncompacted to compacted ratio (>2.0 in adults, >1.4 in children); 4) the absence of another coexisting heart anomaly, including aortic and pulmonary valve abnormalities and coronary disease... The mitral valve affliction is believed to be secondary to the ventricular dysfunction and dilatation caused by INCM... It was treated with beta-blockers and anticoagulant therapy to ensure rhythm control and to prevent clot formation., The increasing number of reported cases of INCM, the high variability of its clinical manifestations, and the diagnosis of INCM in an elderly patient lead us to believe that this disease is actually underestimated... Further research is needed to solve current controversies regarding diagnostic criteria, nomenclature, prognosis, origin and pathogenesis... Some remaining questions include whether or not the presence of congenital heart anomalies should exclude NCM, and whether cases diagnosed in young children or in adults represent subgroups of the disease.

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