Normal left ventricular systolic function in adults with atrial septal defect and left heart failure

Carabello, Blasé A.; Gash, Arnold K.; Mayers, Douglas L.; Spann, James F.

doi:10.1016/0002-9149(82)90204-1

Cited by 32 publications

(16 citation statements)

References 26 publications

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“…This result also explains the fact that the long-term growth of Tbx5 mutants is not affected, probably because the normal systemic cardiac output is maintained. Our results also further conform to the finding in humans that ventricular diastolic dysfunction often precedes the onset of systolic dysfunction and contributes to the symptoms of various cardiac diseases (6,24).…”

Section: Left Ventricular Diastolic Dysfunction In Tbx5 Del/ϩ Micesupporting

confidence: 91%

“…This finding is consistent with that in humans with ASD (3,6). This result also explains the fact that the long-term growth of Tbx5 mutants is not affected, probably because the normal systemic cardiac output is maintained.…”

Section: Left Ventricular Diastolic Dysfunction In Tbx5 Del/ϩ Micesupporting

confidence: 91%

“…In humans with ASD, right ventricular dilatation significantly alters left ventricular geometry (e.g., a flattened inter- ventricular septum toward the left ventricle at end systole and early diastole and decreased left ventricular chamber dimension), increases left ventricular chamber stiffness and reduces its compliance, and consequently impairs left ventricular filling (3,6,17). However, all these abnormalities are not associated with significant changes in the mitral flow pattern in patients with normal pulmonary pressure (21,30,34).…”

Section: Possible Mechanisms For Left Ventricular Diastolic Dysfunctionmentioning

confidence: 99%

“…As seen in humans with right ventricular volume and/or pressure overload caused by ASD, left ventricular diastolic filling is impaired because of right heart dilatation and abnormal interaction of both ventricles (3,6,19,30). Because ASD and resultant right heart dilatation are the major structural anomalies in Tbx5 del/ϩ mice, it is of interest to explore the consequent functional abnormalities of this model compared with those in humans.…”

mentioning

confidence: 99%

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Abnormal cardiac inflow patterns during postnatal development in a mouse model of Holt-Oram syndrome

Zhou¹,

Zhu²,

Bishop³

et al. 2005

American Journal of Physiology-Heart and Circulatory Physiology

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del/ϩ mice showed significant changes in the mitral flow pattern, including decreased peak velocity of the left ventricular (LV) early filling wave (E wave), increased peak velocity of the late filling wave (A wave), and decreased or even reversed peak E-to-A ratio. The prolongation of LV isovolumic relaxation time was detected in Tbx5 del/ϩ neonates as early as 1 wk of age. In Tbx5 del/ϩ mice, LV wall thickness appeared normal but LV chamber dimension was significantly reduced. LV systolic function did not differ from that in wild-type littermates. In contrast, the Doppler flow spectrum in the enlarged tricuspid orifice of Tbx5 del/ϩ mice demonstrated increased peak velocities of both E and A waves and increased total time-velocity integral but unchanged peak E/A. In another 13 mice ( 7 Tbx5 del/ϩ , 6 wild-type) at 2 wk of age, significant correlation was found between Tbx5 gene expression level in ventricular myocardium and LV filling parameters. In conclusion, the LV diastolic function of Tbx5 del/ϩ mice is significantly deteriorated, whereas the systolic function remains normal. Tbx5 mutation; atrial septal defect; left ventricular diastolic function; right ventricular dilatation; Doppler flow sampling HUMAN HOLT-ORAM SYNDROME is an autosomal dominant disease caused by mutations of the TBX5 gene and is characterized by congenital cardiac and forelimb anomalies (1,4,15,16,22). Similar to Holt-Oram syndrome, Tbx5del/ϩ mice demonstrate cardiac anatomic abnormalities including atrial septal defects (ASDs) and/or ventricular septal defects and limb malformations. Secondary changes in cardiac morphology including dilated atria and right ventricle leading to a bulbous shape of the whole heart are observed. Abnormal cardiac electrophysiology, particularly atrioventricular block, has also been found (5, 23).Although the cardiac structural phenotypes of Tbx5 del/ϩ mice have been well identified ex vivo with histology or MRI, the in vivo cardiac functional phenotypes and their comparison with those of corresponding human cardiac disease have not yet been evaluated. As seen in humans with right ventricular volume and/or pressure overload caused by ASD, left ventricular diastolic filling is impaired because of right heart dilatation and abnormal interaction of both ventricles (3,6,19,30). Because ASD and resultant right heart dilatation are the major structural anomalies in Tbx5 del/ϩ mice, it is of interest to explore the consequent functional abnormalities of this model compared with those in humans. Another question is whether Tbx5 mutation that markedly decreases the expression of downstream targeted genes in the developing murine heart (5) affects ventricular myocardial function.Recently, a methodology has been established for comprehensive in vivo cardiac imaging in mice using a high-frequency (ϳ20 -55 MHz) ultrasound imaging, ultrasound biomicroscopy (UBM) (10 -12, 25, 38, 40). This new technology has been successfully applied to measure cardiac dimensions, function, and hemodynamics with various functional modali...

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Section: Left Ventricular Diastolic Dysfunction In Tbx5 Del/ϩ Micesupporting

confidence: 91%

Section: Left Ventricular Diastolic Dysfunction In Tbx5 Del/ϩ Micesupporting

confidence: 91%

Section: Possible Mechanisms For Left Ventricular Diastolic Dysfunctionmentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Abnormal cardiac inflow patterns during postnatal development in a mouse model of Holt-Oram syndrome

Zhou¹,

Zhu²,

Bishop³

et al. 2005

American Journal of Physiology-Heart and Circulatory Physiology

View full text Add to dashboard Cite

show abstract

“…We speculate that in some patients a persistent foramen ovale may have increased in size and thus allowed an overflow secondary to pressure-induced left atrial enlargement. A direct impediment of left ventricular filling by right ventricular volume overload, however, as discussed in other reports [6,7], can be ruled out because volume overload was suppressed by complete balloon occlusion of the septal defect.…”

Section: Discussionmentioning

confidence: 80%

Masked left ventricular restriction in elderly patients with atrial septal defects: A contraindication for closure?

Ewert

Berger

Nagdyman

et al. 2001

Cathet. Cardiovasc. Intervent.

122

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The impact of an atrial septal defect in the elderly with reduced diastolic elasticity of the left ventricle is unclear. We studied the hemodynamic changes during balloon occlusion of atrial septal defects in patients over 60 years of age. In 18 patients (61-78 years old; median, 70), the left atrial pressure and the mitral valve inflow was measured during complete balloon occlusion of the defect and after deflation of the balloon. In seven patients, the left atrial pressure and the E/A ratio of the mitral valve inflow increased markedly (P = 0.02). Mean atrial pressures reached values of 27 mm Hg and the v-wave peak values of 55 mm Hg. Two patients received a transcatheter device closure and developed congestive heart failure. In the elderly, an atrial septal defect can have a decompressive impact on the left ventricle. Therefore, caution appears to be warranted if atrial septal closure is planned.

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Right ventricular diastolic performance: Compliance characteristics with focus on pulmonary hypertension, right ventricular hypertrophy, and calcium channel blockade

Ferlinz

1998

Cathet. Cardiovasc. Diagn.

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Normal left ventricular systolic function in adults with atrial septal defect and left heart failure

Cited by 32 publications

References 26 publications

Abnormal cardiac inflow patterns during postnatal development in a mouse model of Holt-Oram syndrome

Abnormal cardiac inflow patterns during postnatal development in a mouse model of Holt-Oram syndrome

Masked left ventricular restriction in elderly patients with atrial septal defects: A contraindication for closure?

Right ventricular diastolic performance: Compliance characteristics with focus on pulmonary hypertension, right ventricular hypertrophy, and calcium channel blockade

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