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A case of congenital hypertrophic cardiomyopathy.

Seo HS, Lee IH, Song YW, Choi BM, Jang GY, Son CS, Lee JW - Korean Circ J (2013)

Bottom Line: Congenital hypertrophic cardiomyopathy (HCMP) is a very rare congenital heart disease.Here, we report a case of neonatal HCMP, which was confirmed by two-dimensional echocardiography and autopsy.The HCMP rapidly progressed and the patient's condition deteriorated, despite the treatment for congestive heart failure.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Korea University Hospital, Seoul, Korea.

ABSTRACT
Congenital hypertrophic cardiomyopathy (HCMP) is a very rare congenital heart disease. Here, we report a case of neonatal HCMP, which was confirmed by two-dimensional echocardiography and autopsy. The HCMP rapidly progressed and the patient's condition deteriorated, despite the treatment for congestive heart failure.

No MeSH data available.


Related in: MedlinePlus

The echocardiography showed severe hypertrophy of both ventricles and ventricular septum (A) and color Doppler echocardiogram also demonstrated an accelerated flow at the outflow tract of the left ventricle (B).
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Figure 1: The echocardiography showed severe hypertrophy of both ventricles and ventricular septum (A) and color Doppler echocardiogram also demonstrated an accelerated flow at the outflow tract of the left ventricle (B).

Mentions: On the first day after birth, two-dimensional echocardiography demonstrated marked hypertrophy of both ventricles (the ventricular septum was 15-mm-thick and the posterior wall of the left ventricle was 12-mm-thick). Ventilator weaning was not possible and the baby required intermittent positive-pressure ventilation. On the 26th day after birth, two-dimensional echocardiography follow-up demonstrated an increment of ventricular septum and posterior wall thickness of the left ventricle, compared to the first day, showing the ventricular septum with 17-mm-thickness and the posterior wall of the left ventricle with 13-mm-thickness (Fig. 1A). The cavities of the left and right ventricle were small due to the hypertrophied ventricular septum and wall. Narrowing of the outflow tracts in the left and right ventricles resulted in obstruction with pressure gradients measured at 45 mm Hg in the left ventricular outflow tract and 35 mm Hg in the right ventricular outflow tract. The hypertrophy of both ventricles had progressed on the 38th day after birth, and the thicknesses of the ventricular septum and posterior wall of the left ventricle had increased to 21 mm and 15 mm, respectively. A color and pulse Doppler echocardiogram also demonstrated an accelerated flow velocity at the outflow tract of the left ventricle (Fig. 1B). As HCMP was aggravated, left ventricular capacity was decreased gradually, and these brought decreased blood pressure, elevated heart rate and also decreased urine output. Further, as resistance of left atrium was increased, pulmonary congestion was also gradually aggravated. He had no arrhythmia and no infection focus. On the 51st day after birth, despite the administration of maximum dose of inotropics, blood pressure decreased consistently. The heart rate also decreased slowly, finally reaching a rate below 60/min. The infant expired during cardiopulmonary resuscitation without response due to heart failure, and respiratory failure due to pulmonary congestion. An autopsy was performed on the following day after death, showing the thickness of the left and right ventricle wall with about 3.0 cm and 2.0 cm, respectively. Additional abnormality of the heart was not detected, except the already known dextrocardia. No specific findings of the other organs were seen. The microscopic findings of the myocardium indicated myofiber hypertrophy with nuclear enlargement and hyperchromasia. The nuclei predominantly showed a typical box shape (Fig. 2).


A case of congenital hypertrophic cardiomyopathy.

Seo HS, Lee IH, Song YW, Choi BM, Jang GY, Son CS, Lee JW - Korean Circ J (2013)

The echocardiography showed severe hypertrophy of both ventricles and ventricular septum (A) and color Doppler echocardiogram also demonstrated an accelerated flow at the outflow tract of the left ventricle (B).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3569568&req=5

Figure 1: The echocardiography showed severe hypertrophy of both ventricles and ventricular septum (A) and color Doppler echocardiogram also demonstrated an accelerated flow at the outflow tract of the left ventricle (B).
Mentions: On the first day after birth, two-dimensional echocardiography demonstrated marked hypertrophy of both ventricles (the ventricular septum was 15-mm-thick and the posterior wall of the left ventricle was 12-mm-thick). Ventilator weaning was not possible and the baby required intermittent positive-pressure ventilation. On the 26th day after birth, two-dimensional echocardiography follow-up demonstrated an increment of ventricular septum and posterior wall thickness of the left ventricle, compared to the first day, showing the ventricular septum with 17-mm-thickness and the posterior wall of the left ventricle with 13-mm-thickness (Fig. 1A). The cavities of the left and right ventricle were small due to the hypertrophied ventricular septum and wall. Narrowing of the outflow tracts in the left and right ventricles resulted in obstruction with pressure gradients measured at 45 mm Hg in the left ventricular outflow tract and 35 mm Hg in the right ventricular outflow tract. The hypertrophy of both ventricles had progressed on the 38th day after birth, and the thicknesses of the ventricular septum and posterior wall of the left ventricle had increased to 21 mm and 15 mm, respectively. A color and pulse Doppler echocardiogram also demonstrated an accelerated flow velocity at the outflow tract of the left ventricle (Fig. 1B). As HCMP was aggravated, left ventricular capacity was decreased gradually, and these brought decreased blood pressure, elevated heart rate and also decreased urine output. Further, as resistance of left atrium was increased, pulmonary congestion was also gradually aggravated. He had no arrhythmia and no infection focus. On the 51st day after birth, despite the administration of maximum dose of inotropics, blood pressure decreased consistently. The heart rate also decreased slowly, finally reaching a rate below 60/min. The infant expired during cardiopulmonary resuscitation without response due to heart failure, and respiratory failure due to pulmonary congestion. An autopsy was performed on the following day after death, showing the thickness of the left and right ventricle wall with about 3.0 cm and 2.0 cm, respectively. Additional abnormality of the heart was not detected, except the already known dextrocardia. No specific findings of the other organs were seen. The microscopic findings of the myocardium indicated myofiber hypertrophy with nuclear enlargement and hyperchromasia. The nuclei predominantly showed a typical box shape (Fig. 2).

Bottom Line: Congenital hypertrophic cardiomyopathy (HCMP) is a very rare congenital heart disease.Here, we report a case of neonatal HCMP, which was confirmed by two-dimensional echocardiography and autopsy.The HCMP rapidly progressed and the patient's condition deteriorated, despite the treatment for congestive heart failure.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Korea University Hospital, Seoul, Korea.

ABSTRACT
Congenital hypertrophic cardiomyopathy (HCMP) is a very rare congenital heart disease. Here, we report a case of neonatal HCMP, which was confirmed by two-dimensional echocardiography and autopsy. The HCMP rapidly progressed and the patient's condition deteriorated, despite the treatment for congestive heart failure.

No MeSH data available.


Related in: MedlinePlus