[SCD-FORUM] 105E Female patient, 30 years old. Dr. Yunlong Xia

[SCD Symposium]

Dear Dr. Perez Riera and other colleagues

Last week I have met a special case, and here I want to get help from  
you.

Female, 30 years old. She came into my hospital because of black  
spell and syncope for 2 weeks. The ECG shows atrial standstill and  
junctional/ventricular escape rhythm of 36, with atrium suspected to  
be retrograde excited (Fig 1***). After injection of 1 mg atropine  
for 10-20 min, the escape rate was only slightly increased to 50-55  
bpm with standstill atrium (Fig 2***). After that isoprotorenol was  
intravenous infused to keep escape rate 50-55 bpm. Occasionally,  
paroxysmal atrial tachycardia/fibrillation and ventricular premature  
complex could be observed during monitoring, and AV was found not to  
be 1:1 conducted (Fig. 3***, P wave is clear especially on Lead V1).  
The routine blood test, heart enzyme, thyroid test and autoimmune  
system test are all inside normal range. Echocardiography reports the  
significant tricuspid annulus dilation, with tricuspid insufficiency,  
medium pulmonary artery hypertension, intra vena cava dilation (IVC  
20), right system dilation (RA 52 X 61, RV 21), left atrial dilation  
(LA 43 X 57) and normal ventricular wall motion.

After observation for 2 days, the bradycardia continued and a DDDR  
pacemaker was implanted, with atrium lead in right appendage. During  
implantation the atrium was demonstrated to be retrograde activated,  
but no further EP test was performed. After implantation, the P wave  
could be only seen positively on lead V1, whereas in other leads it  
can’t be clearly observed (Fig 4***). Later echocardiography showed  
relatively weak right atrium motion, and left atrium standstill.  
Warfarin was administered to her with INR 1.8-2.5.

SEE ECGs
http://www.scd-symposium.org/files/yunlong1.jpg
http://www.scd-symposium.org/files/yunlong2.jpg
http://www.scd-symposium.org/files/yunlong3.jpg
http://www.scd-symposium.org/files/yunlong4.jpg

Family histories:

Two years ago the lady got pregnant (during that period her ECG is  
normal) and then had a baby boy with normal ECG. She has a younger  
brother and a younger sister with normal ECG now.

The lady’s father has 7 brothers and sisters, in which 4 of them died  
before 40 years old due to heart attack without clear diagnosis,  
because of the limited medical conditions in their hometown. Her  
father and one of her aunt have received pacemaker therapy around 20  
years ago, and the patient did not know their clear diagnosis. Her  
father died from cerebral embolism later and her aunt also died later  
with uncertain reasons.

She don’t know more about her family history now because most of her  
other relatives are scattering in China and living far away from her,  
and cannot be reached so easily. We are still encouraging her to find  
more of her family members.

My question is:

1.     Have you met such kind of familial bradycardia with atrial  
standstill and tricuspid annulus dilation before? Progressive Cardiac  
Conduction Defects (PCCD) or the Lev-Lenègre disease usually causes  
RBBB/LBBB or even complete AVB with sclerosis of the left "cardiac  
skeleton" (Mitral annulus abnormal). However, besides the sinus and/ 
or atrial conduction defect, in this case right “cardiac skeleton”  
seems to be sclerosis.

2.     The patient did not accept the cardiac Computed Tomography due  
to the cost. She doesn’t want EP test and myocardial biopsy now. In  
my hospital we cannot have a genetic test, either. What should we do  
now? If necessary we can encourage the patient to do more test.

3.     After pacemaker implantation, her left atrium is standstill.  
Is it possible that her left atrium function could recover later?


Thanks for your time.

All the best

Yunlong Xia
Department of Cardiology
First Affiliate Hospital of
Dalian Medical University
Dalian, China. 116011
Email: yunlong.xia at gmail.com

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee




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