Acute and short-term effects of partial left ventriculectomy in dilated cardiomyopathy

Schreuder, Jan J.; Steendijk, Paul; Veen, Frederik H. van der; Alfieri, Ottavio; Nagel, Theo van der; Lorusso, Roberto; Dantzig, Jan-Melle van; Prenger, Kees B.; Baan, Jan; Wellens, Hein J.J.; Batista, Randas J.V.

doi:10.1016/s0735-1097(00)01036-6

Cited by 66 publications

(31 citation statements)

References 21 publications

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“…To further establish old mdx mice as a valid model for our study, we performed closed-chest hemodynamic measurement. Consistent with previous reports on dilated cardiomyopathy, 21,22 we observed a marked rightward shift of the PV loop in aged mdx mice ( Figure 3A). Chamber dilation also resulted in significant increase in both end-systolic and end-diastolic volume ( Figure 3B and 3C).…”

Section: Twenty-one-month-old MDX Mice Show Dilated Cardiomyopathysupporting

confidence: 93%

Prevention of Dystrophin-Deficient Cardiomyopathy in Twenty-One-Month-Old Carrier Mice by Mosaic Dystrophin Expression or Complementary Dystrophin/Utrophin Expression

Bostick

Yue

Long

et al. 2008

Circulation Research

107

137

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Abstract-A cure for dystrophin-deficient muscular dystrophy requires treating both skeletal muscle and the heart. Whereas mosaic dystrophin expression has been shown to protect skeletal muscle, controversy exists over whether mosaic expression is protective in the heart. We have shown recently that mosaic dystrophin expression prevents stress-induced heart damage in young carrier mice. Although an interesting finding, the clinical relevance remains to be established because young dystrophin-null mdx mice do not have heart disease. On the other hand, heart failure has been reported in human carriers. To resolve this mouse/human discrepancy, we evaluated the cardiac phenotype in 21-month-old mdx, carrier, and normal mice. We found dilated cardiomyopathy in old mdx mice but not in age-matched carrier mice. All anatomical parameters and physiological assay results (ECG and closed-chest Millar catheter) were within the normal range in old carrier mice. Focal myocardial inflammation was found in a small fraction of old carrier mice, but it had no major impact on heart function. Dobutamine stress revealed a near normal hemodynamic profile except for a marginal reduction in systolic pressure in old carrier mice. Immunostaining and Western blot showed dystrophin expression in 50% cardiomyocytes in old carrier mice. Interestingly, utrophin was upregulated in dystrophin-negative heart cells in carrier mice. In summary, we have provided the first clear-cut evidence that dilated cardiomyopathy in old mdx mice was prevented by mosaic dystrophin expression or complementary dystrophin/utrophin expression. Our results raise the hope for ameliorating dystrophic cardiomyopathy through partial gene and/or cell therapy. Key Words: cardiomyopathy Ⅲ gene therapy Ⅲ myocardium Ⅲ genetics Ⅲ heart disease H eart disease profoundly affects the life quality of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) patients. 1,2 DMD and BMD are caused by mutations in the dystrophin gene. Dystrophin is a long rodshaped cytoskeletal protein located at the cytosolic surface of the sarcolemma. It glues the cytoskeleton, sarcolemma, and extracellular matrix together to prevent cell membrane damage during muscle contraction. Absence of dystrophin or abnormal dystrophin expression weakens the physical link between the extracellular matrix and the cytoskeleton. As a consequence, the affected muscle cells undergo degeneration and necrosis. Eventually, muscle tissue is replaced by fibrous, bony and/or fatty tissue and loses function.Pathology in the heart and the diaphragm determines the life span in DMD/BMD patients. Until recently, approximately 80% to 90% of DMD patients died from respiratory failure because of a weak diaphragm. With improved respiratory care, heart-related death has become more frequent, even approaching 40% in some studies. 3 Currently, symptomatic management is the only treatment option. The advent of gene and cell therapies brings the hope of a cure for DMD/BMD. 2 In gene therapy, the mutated gene is replaced and...

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Section: Twenty-one-month-old MDX Mice Show Dilated Cardiomyopathysupporting

confidence: 93%

Prevention of Dystrophin-Deficient Cardiomyopathy in Twenty-One-Month-Old Carrier Mice by Mosaic Dystrophin Expression or Complementary Dystrophin/Utrophin Expression

Bostick

Yue

Long

et al. 2008

Circulation Research

107

137

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“…Left ventriculoplasty is the surgical reconstruction of the heart as a treatment of end-stage heart failure in patients with DCM introduced by Batista et al [16,17]. The procedure aims to restore the normal relationship between ventricular mass and dimension to normalize wall stress throughout the cardiac cycle.…”

Section: Left Ventriculoplastymentioning

confidence: 99%

High prevalence of chronic myocarditis in dilated cardiomyopathy referred for left ventriculoplasty: expression of tenascin C as a possible marker for inflammation

Tsukada¹,

Terasaki²,

Shimomura³

et al. 2009

Human Pathology

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“…This is in contrast to surgical shape changing procedures, i.e. partial left ventriculectomy, which has been shown to be associated with negative effects on diastolic pressure-volume relationships counteracting the positive effects on systolic improvement [12].…”

Section: Discussionmentioning

confidence: 84%

Echocardiographic findings using tissue velocity imaging following passive containment surgery with the Acorn CorCap™ cardiac support device

Olsson

Bredin

Franco‐Cereceda

2005

European Journal of Cardio-Thoracic Surgery

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Acute and short-term effects of partial left ventriculectomy in dilated cardiomyopathy

Cited by 66 publications

References 21 publications

Prevention of Dystrophin-Deficient Cardiomyopathy in Twenty-One-Month-Old Carrier Mice by Mosaic Dystrophin Expression or Complementary Dystrophin/Utrophin Expression

Prevention of Dystrophin-Deficient Cardiomyopathy in Twenty-One-Month-Old Carrier Mice by Mosaic Dystrophin Expression or Complementary Dystrophin/Utrophin Expression

High prevalence of chronic myocarditis in dilated cardiomyopathy referred for left ventriculoplasty: expression of tenascin C as a possible marker for inflammation

Echocardiographic findings using tissue velocity imaging following passive containment surgery with the Acorn CorCap™ cardiac support device

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