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[Diastolic dysfunction and left ventricular hypertrophy in familial amyloidotic polyneuropathy: a cause-effect relationship?].
Rev Port Cardiol. 2000 Apr; 19(4):477-81.RP

Abstract

TTR Met30 Familial Amyloidotic Polyneuropathy of the Portuguese type (FAP) is an incapacitating and lethal hereditary disorder that affects predominantly young adults of both genders. Portuguese type FAP patients have sensory, motor and autonomic polyneuropathy. The generalised systemic amyloid infiltration involves the heart, leading to the characteristic granular bright sparkling echocardiographic pattern. LV wall thickening occurs in the late phases of the disease. LV diastolic dysfunction has been reported in the absence of systolic dysfunction; an abnormal diastolic transmitral flow pattern assessed by pulsed wave Doppler (PW) was described. PW is very much dependent on load conditions. Tissue Doppler imaging (TDI) has been used as a more reliable method to assess long axis diastolic function.

OBJECTIVE

1--To identify the incremental value of TDI in the assessment of diastolic function in FAP. 2--To correlate diastolic pattern abnormalities and left ventricular mass index (LVMI) in FAP patients.

METHODS

We performed a prospective evaluation of 24 consecutive FAP patients and selected 14 (sinus rhythm, age < 45 years). Diastolic function was assessed by PW and classified as normal (GI-E/A > 1) or abnormal (GII-E/A < 1). TDI was performed in 4 sites of the mitral annulus (septum, lateral, inferior, anterior). Velocities of the rapid filling wave (E') and atrial contraction wave (A') were measured and E'/A' calculated. In each site we considered the TDI as normal (E'/A' > 1) or abnormal (E'/A' < 1). The LVMI was calculated by Devereux's formula.

RESULTS

Age, gender and heart rate were similar in both groups. TDI at the septal mitral annulus was normal in all of the GI patients (E'/A': 1.29 +/- 0.19) and suggestive of abnormal LV relaxation in all of the GII patients (E'/A': 0.82 +/- 0.11, p < 0.0001). TDI revealed abnormal diastolic pattern when a restricted number of sites of the mitral annulus were assessed, even in GI patients and before PW abnormalities occurred. Fractional shortening (FS) and LVMI were similar in GI and GII (FS-GI: 45.5 +/- 5.3, GII 43.5 +/- 8.1%, p: NS; LVMI--GI: 66 +/- 9.3, GII: 67 +/- 3.0 g/m2 p: NS).

CONCLUSION

The assessment of mitral annulus motion has introduced new data in the study of diastolic function of FAP patients. An abnormal LV relaxation pattern occurred early in the evolution of the disease in patients with normal LVMI and systolic function.

Authors+Show Affiliations

Serviço de Cardiologia do Hospital Pulido Valente.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

por

PubMed ID

10874844

Citation

Fonseca, C, et al. "[Diastolic Dysfunction and Left Ventricular Hypertrophy in Familial Amyloidotic Polyneuropathy: a Cause-effect Relationship?]." Revista Portuguesa De Cardiologia : Orgao Oficial Da Sociedade Portuguesa De Cardiologia = Portuguese Journal of Cardiology : an Official Journal of the Portuguese Society of Cardiology, vol. 19, no. 4, 2000, pp. 477-81.
Fonseca C, Cardim N, Morais H, et al. [Diastolic dysfunction and left ventricular hypertrophy in familial amyloidotic polyneuropathy: a cause-effect relationship?]. Rev Port Cardiol. 2000;19(4):477-81.
Fonseca, C., Cardim, N., Morais, H., Ferreira, T., Pereira, A. T., Luís, M. L., Luís, A. S., Ceia, F., & Correia, J. M. (2000). [Diastolic dysfunction and left ventricular hypertrophy in familial amyloidotic polyneuropathy: a cause-effect relationship?]. Revista Portuguesa De Cardiologia : Orgao Oficial Da Sociedade Portuguesa De Cardiologia = Portuguese Journal of Cardiology : an Official Journal of the Portuguese Society of Cardiology, 19(4), 477-81.
Fonseca C, et al. [Diastolic Dysfunction and Left Ventricular Hypertrophy in Familial Amyloidotic Polyneuropathy: a Cause-effect Relationship?]. Rev Port Cardiol. 2000;19(4):477-81. PubMed PMID: 10874844.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Diastolic dysfunction and left ventricular hypertrophy in familial amyloidotic polyneuropathy: a cause-effect relationship?]. AU - Fonseca,C, AU - Cardim,N, AU - Morais,H, AU - Ferreira,T, AU - Pereira,A T, AU - Luís,M L, AU - Luís,A S, AU - Ceia,F, AU - Correia,J M, PY - 2000/6/30/pubmed PY - 2000/7/25/medline PY - 2000/6/30/entrez SP - 477 EP - 81 JF - Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology JO - Rev Port Cardiol VL - 19 IS - 4 N2 - UNLABELLED: TTR Met30 Familial Amyloidotic Polyneuropathy of the Portuguese type (FAP) is an incapacitating and lethal hereditary disorder that affects predominantly young adults of both genders. Portuguese type FAP patients have sensory, motor and autonomic polyneuropathy. The generalised systemic amyloid infiltration involves the heart, leading to the characteristic granular bright sparkling echocardiographic pattern. LV wall thickening occurs in the late phases of the disease. LV diastolic dysfunction has been reported in the absence of systolic dysfunction; an abnormal diastolic transmitral flow pattern assessed by pulsed wave Doppler (PW) was described. PW is very much dependent on load conditions. Tissue Doppler imaging (TDI) has been used as a more reliable method to assess long axis diastolic function. OBJECTIVE: 1--To identify the incremental value of TDI in the assessment of diastolic function in FAP. 2--To correlate diastolic pattern abnormalities and left ventricular mass index (LVMI) in FAP patients. METHODS: We performed a prospective evaluation of 24 consecutive FAP patients and selected 14 (sinus rhythm, age < 45 years). Diastolic function was assessed by PW and classified as normal (GI-E/A > 1) or abnormal (GII-E/A < 1). TDI was performed in 4 sites of the mitral annulus (septum, lateral, inferior, anterior). Velocities of the rapid filling wave (E') and atrial contraction wave (A') were measured and E'/A' calculated. In each site we considered the TDI as normal (E'/A' > 1) or abnormal (E'/A' < 1). The LVMI was calculated by Devereux's formula. RESULTS: Age, gender and heart rate were similar in both groups. TDI at the septal mitral annulus was normal in all of the GI patients (E'/A': 1.29 +/- 0.19) and suggestive of abnormal LV relaxation in all of the GII patients (E'/A': 0.82 +/- 0.11, p < 0.0001). TDI revealed abnormal diastolic pattern when a restricted number of sites of the mitral annulus were assessed, even in GI patients and before PW abnormalities occurred. Fractional shortening (FS) and LVMI were similar in GI and GII (FS-GI: 45.5 +/- 5.3, GII 43.5 +/- 8.1%, p: NS; LVMI--GI: 66 +/- 9.3, GII: 67 +/- 3.0 g/m2 p: NS). CONCLUSION: The assessment of mitral annulus motion has introduced new data in the study of diastolic function of FAP patients. An abnormal LV relaxation pattern occurred early in the evolution of the disease in patients with normal LVMI and systolic function. SN - 0870-2551 UR - https://www.unboundmedicine.com/medline/citation/10874844/[Diastolic_dysfunction_and_left_ventricular_hypertrophy_in_familial_amyloidotic_polyneuropathy:_a_cause_effect_relationship]_ L2 - https://antibodies.cancer.gov/detail/CPTC-TTR-1 DB - PRIME DP - Unbound Medicine ER -