[SCD-FORUM] 36E RE: 一例肥厚型心肌病失代偿期（burned-out phase）的女性患者 Dr. Furlani

[SCD Symposium]

36E RE: 一例肥厚型心肌病失代偿期（burned-out phase）的 
女性患者   Dr. Furlani

亲爱的Alizadeh教授：
这真是一个有趣的病例！同时这也是一个困难的病例。

这个案例听上去像是家族性扩张型心肌病或是肥厚型 
心肌病终末扩张期，但是我希望能有更多的资料。她 
的左室射血分数究竟是多少？她的心超是否提示左室 
壁活动异常？她有没有典型的胸痛？她的胆固醇水平 
如何？（家族性的血脂异常可以解释这个患者早发的 
缺血性心脏病。）

她有没有高血压病史？我的意思是说，尽管继发于高 
血压的左室肥厚通常是均匀的，有时严重的高血压也 
可以造成不对称的左室肥厚。假定没有明显的左心瓣 
膜疾病，严重的右室功能障碍和中重度肺动脉高压 
（PAP 65-70mmHg）的出现提示该患者有长期存在的严重 
的左室功能不全，而且预后不好（对CRT反应不好）。 
而且，如果这个患者有继发于严重左室扩张的重度二 
尖瓣反流，CRT对她的益处也不大。

关于药物治疗，我认为予以小剂量的利尿剂和ACEI， 
但不加用β受体阻滞剂和醛固酮抑制剂。在植入起搏 
器之后给予的优化药物治疗要包括剂量合适的ACEI、β 
受体阻滞剂和醛固酮抑制剂，这些药物可能会改善左 
室收缩功能而不需要ICD/CRT的治疗。

最后，她有一个明确的永久起搏器植入指征（完全的 
房室传导阻滞伴宽QRS，不提示his束下阻滞）。右室心 
尖部起搏会诱发左室的不同步，接着很可能会导致左 
室收缩功能和充血性心衰症状的恶化，而右室间隔上 
部的起搏会减少发生左室收缩不同步的可能性。

总之，如果她的LVEF超过30%，我建议植入DDD起搏器并 
把电极固定在右室间隔上部，再加上优化的药物治疗 
（ACEI，BB，醛固酮抑制剂）。然而，如果她的LVEF明 
显低于30%（最好是用核素心室造影），我建议没有重 
度二尖瓣反流就植入CRT-ICD，或者如果有重度二尖瓣 
反流就植入DDD-ICD（通常是作为心脏移植的过渡）。 
因为这例患者CRT的反应不好，就使她成为了心脏移植 
的良好适应征患者。

感谢大家。

感谢大会组织者。


Aldo Alberto Furlani MD
Cardiac Electrophysilogist
Consultant Cardiologist
Heart Institute of the Caribbean
afurlani at caribbeanheart.com
www.caribbeanheart.com


Dear Dr Alizadeh:
What an interesting case! A difficult one too.
It sounds like familial dilated cardiomyopathy versus final dilated
phase of hypertrophic cardiomyopathy, but I would like to have more
information. What exactly is her LV ejection fraction?  Were there
any LV wall motion abnormalities in her Echo? Has she ever had
typical chest pain? What about her cholesterol levels? (a familial
dyslipemia could explain an early onset of ischaemic heart disease in
this patient).
Does she have a history of hypertension? I mean, even though LV
hypertrophy secondary to hypertension is usually concentric,
sometimes severe hypertension could lead to asymetric LV hypertrophy.
Assuming there is no any significant left sided valvular disease, the
presence of severe RV dysfunction and moderate to severe pulmonary
hypertension (PAPS 65-70 mm Hg) implies long standing severe LV
dysfunction in this patient and usually poor prognosis (and lack of
response to CRT). Moreover, if this patient has severe mitral
regurgitation secondary to severe LV dilatation CRT is unlikely to
benefit her.
Regarding her medical treatment, I assume she is on low dose
diuretics and ACEI but she is off beta blockers and anti-aldosterone
drugs. After pacemaker implantation, an optimize medical treatment
including appropriate dose of ACEI, beta-blockers and anti-
aldosterone drugs will probably improve her LVsystolic function
rendering unnecessary ICD/CRT therapy.
Finally, There is a clear indication for permanent pacemaker
implantation (complete AV block with a wide QRS complex escape
suggesting infra-hisian block). RV apical pacing will induce, LV
dyssynchony that, in turn, will probably lead to worsening of both LV
systolic function and CHF symptoms, but high septal RV pacing is less
likely to cause severe LV dyssynchrony.
In summary, If her LVEF is over 30% I will go for a DDD pacemaker
with an active fixation lead in the high RV septum plus optimization
of medical treatment (ACEI, BB, anti-aldost). However, if her LVEF is
clearly below 30% (by nuclear ventriculography ideally) I will
implant either a CRT-ICD in the case of lack of severe mitral
regurgitation or DDD-ICD (usually as a bridge to cardiac
transplantation) when severe MR is present. The lack of response to
CRT will make this young patient a good candidate to cardiac
transplantation.
Thank you all.
Congratulations to the organizers.

Aldo Alberto Furlani MD
Cardiac Electrophysilogist
Consultant Cardiologist
Heart Institute of the Caribbean
afurlani at caribbeanheart.com
www.caribbeanheart.com

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee