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A case of mid-apical obstructive hypertrophic cardiomyopathy treated with a transapical myectomy approach: a case report.

Scudeler TL, Rezende PC, Oikawa FT, da Costa LM, Hueb AC, Hueb W - J Med Case Rep (2014)

Bottom Line: A 63-year-old Caucasian woman presented with a history of chest pain and shortness of breath causing significant limitations on her daily life activities.Transthoracic echocardiography revealed normal systolic function and significant concentric left ventricular hypertrophy that was greater in the mid-apical region.No complications occurred, and her symptoms resolved.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Atherosclerosis, Heart Institute (InCor), University of São Paulo, Avenida Doutor Enéas de Carvalho Aguiar 44, AB, Sala 114, Cerqueira César, 05403-000 São Paulo, SP, Brazil. whady.hueb@incor.usp.br.

ABSTRACT

Introduction: Hypertrophic cardiomyopathy is a genetic cardiac disease characterized by marked variability in morphological expression and natural history. The hypertrophic myocardium is often confined to the septum or lateral wall of the left ventricle, but it can also be encountered in the middle or apical segments of the myocardium. Treatment is based on medical therapy. Others therapies, such as embolization of the septal artery or ventriculomyectomy, are indicated in special situations. Surgery is the standard treatment, and it is classically done via a transaortic approach; however, in cases in which the hypertrophic myocardium is confined to mid-apical segments, a transapical approach is an option. Only a few cases of mid-apical obstructive hypertrophic cardiomyopathy treated with a myectomy using a transapical approach have been reported in the English-language literature. In this report, we present a case of a patient with mid-apical obstructive hypertrophic cardiomyopathy treated using this new approach.

Case presentation: A 63-year-old Caucasian woman presented with a history of chest pain and shortness of breath causing significant limitations on her daily life activities. She had a history of coronary artery disease. Her physical examination was unremarkable. Transthoracic echocardiography revealed normal systolic function and significant concentric left ventricular hypertrophy that was greater in the mid-apical region. Nuclear magnetic resonance imaging confirmed significant hypertrophy of the median segments of the left ventricle. The patient had persistent symptoms despite receiving optimized medical treatment, and a surgical approach was indicated. As a myectomy using transaortic technique was thought to be difficult to perform in her case, a transapical approach was used. No complications occurred, and her symptoms resolved.

Conclusion: A transapical myectomy should be taken into consideration for patients with mid-apical obstructive hypertrophic cardiomyopathy that is refractory to medical treatment.

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Intraoperative photographs illustrating the transapical approach. (A) The patient’s apical hypertrophy before myectomy. (B) Augmentation of the left ventricular cavity after myectomy.
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Figure 4: Intraoperative photographs illustrating the transapical approach. (A) The patient’s apical hypertrophy before myectomy. (B) Augmentation of the left ventricular cavity after myectomy.

Mentions: A 63-year-old Caucasian woman presented with a history of chest pain (Canadian Cardiovascular Society (CCS) class II) and shortness of breath that had progressively worsened with ordinary exertion during the previous three months (New York Heart Association (NYHA) grade III) and had caused significant limitations on her activities of daily living. She reported hypertension, diabetes mellitus, dyslipidemia and obesity grade 1 (World Health Organization classification). She also had a history of unstable angina for which she had received medical treatment in 2004, when an angiogram revealed that she had occlusion of the distal portion of the anterior descending artery. She had a new episode in 2008, when she underwent angioplasty with a stent in the circumflex artery. After this surgery, she was asymptomatic until July 2010, when she returned to the hospital because of episodes of chest pain associated with dyspnea during moderate exertion. Her physical examination at that time was unremarkable, and medical treatment was optimized. Transthoracic echocardiography revealed normal systolic function (ejection fraction=0.75) but significant concentric left ventricular hypertrophy that was greater in the mid-apical region (Figure 1). Her intra-ventricular gradient was 36mm Hg. Nuclear magnetic resonance (NMR) imaging confirmed significant hypertrophy of the median segments of the LV, including 24mm of the medial portion of the inferoseptal segment, that was causing obstruction of the ventricular cavity during systole (Figure 2A). A coronary angiogram revealed chronic occlusion of the anterior descending artery and stent in the circumflex artery free of obstructions. A left-side ventriculogram demonstrated significant hypertrophy in the mid-apical region with apical dyskinesia (Figure 3) and an intraventricular gradient of 100mm Hg. The case was discussed with the cardiac surgery team. Because a myectomy with a transaortic technique was thought to be difficult to perform in her case because of her mid-apical hypertrophy, the procedure was performed using a transapical approach (Figure 4).In the follow-up beyond 30 days, the patient’s NYHA class improved from III to I, and her CCS class improved from II to I. Furthermore, the medication being used was significantly reduced postoperatively (from seven to four). New NMR imaging was performed after the surgery, and it confirmed the disappearance of the mid-ventricular pressure gradient. Additionally, there was a reduction in the total myocardial septum mass from 220g to 162g (Figure 2B).


A case of mid-apical obstructive hypertrophic cardiomyopathy treated with a transapical myectomy approach: a case report.

Scudeler TL, Rezende PC, Oikawa FT, da Costa LM, Hueb AC, Hueb W - J Med Case Rep (2014)

Intraoperative photographs illustrating the transapical approach. (A) The patient’s apical hypertrophy before myectomy. (B) Augmentation of the left ventricular cavity after myectomy.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4232228&req=5

Figure 4: Intraoperative photographs illustrating the transapical approach. (A) The patient’s apical hypertrophy before myectomy. (B) Augmentation of the left ventricular cavity after myectomy.
Mentions: A 63-year-old Caucasian woman presented with a history of chest pain (Canadian Cardiovascular Society (CCS) class II) and shortness of breath that had progressively worsened with ordinary exertion during the previous three months (New York Heart Association (NYHA) grade III) and had caused significant limitations on her activities of daily living. She reported hypertension, diabetes mellitus, dyslipidemia and obesity grade 1 (World Health Organization classification). She also had a history of unstable angina for which she had received medical treatment in 2004, when an angiogram revealed that she had occlusion of the distal portion of the anterior descending artery. She had a new episode in 2008, when she underwent angioplasty with a stent in the circumflex artery. After this surgery, she was asymptomatic until July 2010, when she returned to the hospital because of episodes of chest pain associated with dyspnea during moderate exertion. Her physical examination at that time was unremarkable, and medical treatment was optimized. Transthoracic echocardiography revealed normal systolic function (ejection fraction=0.75) but significant concentric left ventricular hypertrophy that was greater in the mid-apical region (Figure 1). Her intra-ventricular gradient was 36mm Hg. Nuclear magnetic resonance (NMR) imaging confirmed significant hypertrophy of the median segments of the LV, including 24mm of the medial portion of the inferoseptal segment, that was causing obstruction of the ventricular cavity during systole (Figure 2A). A coronary angiogram revealed chronic occlusion of the anterior descending artery and stent in the circumflex artery free of obstructions. A left-side ventriculogram demonstrated significant hypertrophy in the mid-apical region with apical dyskinesia (Figure 3) and an intraventricular gradient of 100mm Hg. The case was discussed with the cardiac surgery team. Because a myectomy with a transaortic technique was thought to be difficult to perform in her case because of her mid-apical hypertrophy, the procedure was performed using a transapical approach (Figure 4).In the follow-up beyond 30 days, the patient’s NYHA class improved from III to I, and her CCS class improved from II to I. Furthermore, the medication being used was significantly reduced postoperatively (from seven to four). New NMR imaging was performed after the surgery, and it confirmed the disappearance of the mid-ventricular pressure gradient. Additionally, there was a reduction in the total myocardial septum mass from 220g to 162g (Figure 2B).

Bottom Line: A 63-year-old Caucasian woman presented with a history of chest pain and shortness of breath causing significant limitations on her daily life activities.Transthoracic echocardiography revealed normal systolic function and significant concentric left ventricular hypertrophy that was greater in the mid-apical region.No complications occurred, and her symptoms resolved.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Atherosclerosis, Heart Institute (InCor), University of São Paulo, Avenida Doutor Enéas de Carvalho Aguiar 44, AB, Sala 114, Cerqueira César, 05403-000 São Paulo, SP, Brazil. whady.hueb@incor.usp.br.

ABSTRACT

Introduction: Hypertrophic cardiomyopathy is a genetic cardiac disease characterized by marked variability in morphological expression and natural history. The hypertrophic myocardium is often confined to the septum or lateral wall of the left ventricle, but it can also be encountered in the middle or apical segments of the myocardium. Treatment is based on medical therapy. Others therapies, such as embolization of the septal artery or ventriculomyectomy, are indicated in special situations. Surgery is the standard treatment, and it is classically done via a transaortic approach; however, in cases in which the hypertrophic myocardium is confined to mid-apical segments, a transapical approach is an option. Only a few cases of mid-apical obstructive hypertrophic cardiomyopathy treated with a myectomy using a transapical approach have been reported in the English-language literature. In this report, we present a case of a patient with mid-apical obstructive hypertrophic cardiomyopathy treated using this new approach.

Case presentation: A 63-year-old Caucasian woman presented with a history of chest pain and shortness of breath causing significant limitations on her daily life activities. She had a history of coronary artery disease. Her physical examination was unremarkable. Transthoracic echocardiography revealed normal systolic function and significant concentric left ventricular hypertrophy that was greater in the mid-apical region. Nuclear magnetic resonance imaging confirmed significant hypertrophy of the median segments of the left ventricle. The patient had persistent symptoms despite receiving optimized medical treatment, and a surgical approach was indicated. As a myectomy using transaortic technique was thought to be difficult to perform in her case, a transapical approach was used. No complications occurred, and her symptoms resolved.

Conclusion: A transapical myectomy should be taken into consideration for patients with mid-apical obstructive hypertrophic cardiomyopathy that is refractory to medical treatment.

Show MeSH
Related in: MedlinePlus