Right ventricular diastolic function in patients with thalassemia major

LETTER TO THE EDITOR

Right ventricular diastolic function in patients with thalassemia major

Sait Demirkol^I; Sevket Balta^I; Mustafa Cakar^II

^IGulhane Military Medical Academy, School of Medicine, Department of Cardiology, Ankara, Turkey

^IIGulhane Military Medical Academy, School of Medicine, Department of Internal Medicine, Ankara, Turkey

^{Mailing Address}

Keywords: Ventricular Function, Right; beta-Thalassemia; Echocardiography.

Dear Editor,

We read with great interest the article "Echocardiography in Thalassemic Patients on Blood Transfusions and Chelation without Heart Failure" written by Rodrigues et al¹. They aimed to evaluate cardiac structure and function by conventional Doppler echocardiography and tissue Doppler imaging in patients with thalassemia major (TM) and no clinical evidence of heart failure. They found that left atrium volume index, mitral septal E/Em ratio and duration of reverse pulmonary vein flow were significantly higher in TM than in anemia and healthy groups. However, no differences were found in left and right ventricular structures and systolic function indexes between the groups. We believe that these findings will act as a guide for further studies regarding echocardiographic evaluation of left and right ventricular functions in patients with TM.

Contrary to common belief, the right ventricle is not a passive chamber. A large number of acute and chronic diseases have been associated with RV diastolic dysfunction, including both pressure and volume overload pathologies such as primary lung disease, congenital heart disease, cardiomyopathies, ischemic heart disease, systemic diseases^2,3. Only a few studies assessed the RV diastolic function. In those studies, the RV diastolic function has been evaluated using Doppler velocities of the transtricuspid flow (E, A, and E/A), tissue Doppler velocities of the tricuspid annulus (E', A', E'/A'), deceleration time, and IVRT. Most commonly used in recent studies are the tricuspid E/E' ratio, RA area or volume, and diastolic strain rate. A good correlation was shown between the tricuspid E/E' ratio and RA volume, and hemodynamic parameters. An E/E' ratio >4 had high sensitivity and specificity for predicting RA pressure >10 mm Hg in non-cardiac surgery intensive care unit patients⁴. The current study¹ did not evaluate RV diastolic function and RA volume. Further studies should be conducted to assess RA volume and RV tissue Doppler diastolic parameters such as tricuspid E/E' ratio.

References

Manuscript received December 29, 2012; manuscript revised December 29, 2012; manuscript accepted 15, 2013.

Reply

In response to the letter, we appreciate the interest shown and comments made on our work.¹ We hope that our data will stimulate new research especially in the field of biventricular diastolic function in individuals with thalassemia major.

We fully agree with comments in the letter on cardiac right chambers evaluation. According to Efthimiadis et al², patients with thalassemia major and right ventricular (RV) restrictive filling pattern had a 15-year cumulative survival rate of 34%, whereas in those without restrictive pattern, survival rate was 82%. Iron deposition is the main cause of cardiac injuries and can affect all structures, including papillary muscles, conduction system, pericardium, and all cardiac cavities. However, as the left ventricular free wall epicardium is the most affected site³, the left ventricle is the cavity generally evaluated.

We would like to take this opportunity to state that we have analyzed some echocardiographic parameters related to RV systolic and diastolic functions in our patients (unpublished data), but we have failed to demonstrate any significant difference between groups (Thalassemia, Healthy, and Anemia). These parameters include ejection fraction, Tei index, tricuspid annulus plane systolic excursion, and tissue Doppler velocities of the tricuspid annulus (S', E', A', and E'/A'). We believe that new techniques for evaluating ventricular and atrial functions, such as speckle tracking, strain, and strain rate^4-6 will be useful in future studies of patients with thalassemia major to identify early signs of right heart structural and functional changes.

Sincerely,

Alexandre Rodrigues,

Fabio Villaça Guimarães-Filho,

João Carlos Ferreira Braga,

Cássia Spínola Custódio Rodrigues,

Paulo Waib,

Antonio Fabron-Junior,

Doralice Marvulle Tan,

Ana Cristina C V França,

Marina Politi Okoshi,

Katashi Okoshi.

References

Correspondência:

Sait Demirkol

Department of Cardiology - Gulhane School of Medicine

Tevfik Saglam St.

Postal Code 06018, Etlik - Ankara, Turquia

E-mail: [e-mail]

Artigo recebido em 29/12/12; revisado em 29/12/12; aceito em 15/02/13.

Carta-resposta

Em resposta à carta, agradecemos o interesse demonstrado e os comentários feitos sobre o nosso trabalho¹. Esperamos que os nossos dados estimulem novas pesquisas, principalmente no campo da função diastólica biventricular em indivíduos com talassemia major.

Concordamos plenamente com os comentários apresentados na carta sobre a avaliação das câmaras direitas cardíacas. De acordo com Efthimiadis e cols.², pacientes com talassemia major e padrão de enchimento restritivo ventricular direito (VD) tiveram taxa de sobrevivência cumulativa de 15 anos de 34%, enquanto que aqueles sem padrão restritivo tiveram taxa de sobrevivência de 82%. A deposição de ferro é a principal causa de lesões cardíacas, podendo afetar todas as estruturas, incluindo os músculos papilares, o sistema de condução, o pericárdio e todas as cavidades cardíacas. No entanto, como o epicárdio da parede livre do ventrículo esquerdo é o local mais afetado³, o ventrículo esquerdo é a cavidade geralmente avaliada.

Gostaríamos de aproveitar esta oportunidade para afirmar que analisamos alguns parâmetros ecocardiográficos relacionados à função sistólica e diastólica do VD em nossos pacientes (dados não publicados), mas não conseguimos demonstrar diferenças significativas entre os grupos (grupo talassêmico, saudável e com anemia). Esses parâmetros incluem fração de ejeção, índice Tei, excursão sistólica do plano do anel da valva tricúspide e as velocidades do Doppler tecidual do anel da valva tricúspide (S', E', A' e E'/A'). Acreditamos que novas técnicas de avaliação das funções ventriculares e atriais, como speckle tracking, strain e strain rate^4-6 serão úteis em futuros estudos com pacientes com talassemia major para identificar sinais precoces de alterações cardíacas direitas estruturais e funcionais.

Atenciosamente,

Alexandre Rodrigues,

Fabio Villaça Guimarães-Filho,

João Carlos Ferreira Braga,

Cássia Spínola Custódio Rodrigues,

Paulo Waib,

Antonio Fabron-Junior,

Doralice Marvulle Tan,

Ana Cristina C V França,

Marina Politi Okoshi,

Katashi Okoshi.

Referências

• 1. Rodrigues A, Guimarães-Filho FV, Braga JC, Rodrigues CS, Waib P, Fabron-Junior A, et al. Echocardiography in thalassemic patients on blood transfusions and chelation without heart failure. Arq Bras Cardiol. 2013;100(1):75-81.
• 2. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K, et al. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr. 2010;23(7):685-713.
• 3. Cardoso SM, Miyague NI. Right ventricular diastolic dysfunction in the postoperative period of tetralogy of Fallot. Arq Bras Cardiol. 2003;80(2):198-201, 194-7.
• 4. Sade LE, Gulmez O, Eroglu S, Sezgin A, Muderrisoglu H. Noninvasive estimation of right ventricular filling pressure by ratio of early tricuspid inflow to annular diastolic velocity in patients with and without recent cardiac surgery. J Am Soc Echocardiogr. 2007;20(8):982-8.
• 1. Rodrigues A, Guimarães-Filho FV, Braga JC, Rodrigues CS, Waib P, Fabron-Junior A, et al. Echocardiography in thalassemic patients on blood transfusions and chelation without heart failure. Arq Bras Cardiol. 2013;100(1):75-81.
• 2. Efthimiadis GK, Giannakoulas G, Hassapopoulou HP, Karvounis HI, Tsikaderis DD, Styliadis IH, et al. Prognostic significance of right ventricular diastolic function in thalassaemia major. Swiss Med Wkly. 2008;138(3-4):52-4.
• 3. Aessopos A, Berdoukas V, Tsironi M. The heart in transfusion dependent homozygous thalassaemia today - prediction, prevention and management. Eur J Haematol. 2008;80(2):93-106.
• 4. Ojaghi Haghighi Z, Naderi N, Amin A, Taghavi S, Sadeghi M, Moladoust H, et al. Quantitative assessment of right atrial function by strain and strain rate imaging in patients with heart failure. Acta Cardiol. 2011;66(6):737-42.
• 5. Motoki H, Dahiya A, Bhargava M, Wazni OM, Saliba WI, Marwick TH, et al. Assessment of left atrial mechanics in patients with atrial fibrillation: comparison between two-dimensional speckle-based strain and velocity vector imaging. J Am Soc Echocardiogr. 2012;25(4):428-35.
• 6. Altekin RE, Yanikoglu A, Karakas MS, Ozel D, Kucuk M, Yilmaz H, et al. Assessment of left atrial dysfunction in obstructive sleep apnea patients with the two dimensional speckle-tracking echocardiography. Clin Res Cardiol. 2012;101(6):403-13.

Publication Dates

• Publication in this collection
  31 July 2013
• Date of issue
  July 2013