3/31/2015
CASE PRESENTATION: :
Presented by:
Dr. Khalid Amin Attas HOD/Consultant Pediatric Cardiologist Um Al Qura Cardiac Center Maternity and Children’s Hospital, Makkah
History:
Tahani is 16 years old female adolescent, diagnosed to have D-TGA in Yemen came with a complain of increasing shortness of breath with daily task and increasing cyanosis with daily activity.
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On examination:
Patient looks bluish with clopping finger, oxygen saturation was 60 % on room air, HR was 94 b/min., BP was 100/66 (97) mmHg, Wt. 27.5 kg.
Heart: apex with 6th ICS, outside MCL. S1+S2+0. Abdomen: NAD Chest x-ray:
Caridiomegaly, increase lung vascularity.
ECG:
Sinus rhythm, right atrial enlargement.
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Echocardiography:
Situs solitus, levocardia, normal systemic and pulmonary venous connections, AV concordance, VA discordance, D-TGA, dilated pulmonary artery, aorta anterior and to the right, ASD II measured 1 cm with bidirectional shunt, no RVOT or LVOT obstruction, left sided aortic arch, no coarctation, no PDA, no coarctation, good myocardial function.
First Cardiac catheterization on (11-11-2006 / 20-10-1427): HEMODYNAMICS: in room air O2 saturation (%) SVC IVC RA RV MPA PV upper LA LV Asc. Aorta RFA
Cond. 1 55% 50% 58% 73% 87% --86% 84% 63% ---
Cond. 2 52% --63% --91% 99% 92% 92% 73% ---
Pressure (mmHg) Cond. 1 ----8/8/5 104/6/12 79/43/55 --10/8/6 77/6/9 --106/6/78
Cond. 2 ----11/9/7 --70/51/58 --9/8/7 77/6/11 91/64/76 ---
Estimated O2 Consumption: Wt.: 27 kg.
191.3 ml/min/m2 Height: 147 cm BSA: 1.09 m² Hb: 22 g/dl
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Calculation Qp: Qs: Qp/Qs:
Cond. 1 6.87 6.88 1.00
Cond. 2 7.52 3.03 2.48
Calculation TPR PAR Index SVR Index
Cond. 1 8.0 7.7 11.5
Cond. 2 7.7 7.4 24.7
ABGs: Condition Cond. 1 Cond. 2
PH 7.335 7.338
PO2 35 43
PCO2 41.1 40.1
HCO3 20.5 20.6
O2 saturation 63% 75.9%
Angiographic Findings:
Angiography to the left ventricle showed good size left ventricle, intact interventricular septum, Aortic root injection showed left side aortic arch, no coarctation, no aortic incompetence, no PDA.
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Assessment:
A 16 years old female adolescent with, D-TGA and pulmonary hypertension.
Plan:
In view of the above-mentioned data, this patient is was discharged home on oxygen and Sildenafil 25 mg BID, Lasix 20 mg once daily and Aspirin 81 mg once daily and the patient given a follow-up after 6 months.
Second Cardiac catheterization on
(24/9/2007-12/9/1428):
HEMODYNAMICS: in room air O2 saturation (%) SVC IVC RA RV MPA LUPV LA LV Asc. Aorta FA
Cond. I 47% 55% --61% 83% 90% 93% --70% ---
Cond. II 60.5% 78% ----97% 99.7% ----88% ---
Pressure (mmHg) Cond. I ----9/8/5 127/14 84/52/66 --7/8/6 96/3 128/91/108 111/71/87
Cond. II ----11/11/8 --74/49/59 --5/7/8 77/13 134/90/106 ---
Estimated O2 Consumption: 198 ml/min/m2 Wt.: 33 kg Height: 148 cm BSA: 1.19 m² Hb: 18.4 g/dl
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ABGs: Calculation Qp: Qs:
Cond. I 11.1 3.4
Cond. II 19 3.4
3.2
5.7
Qp/Qs:
Condition Cond. I
PH 7.34
PO2 38.6
PCO2 40.2
Calculation PVR PAR Index SVRI PVR/SVR
HCO3 20.6
Cond. I 5.4 w.u. 6.4 w.u. 28.6 0.2
Cond. II 2.7 w.u. 3.2 w.u. 34.6 0.1
O2 saturation 70
Angiographic Findings :
Angiography to the left ventricle showed good size left ventricle, intact interventricular septum, Aortic root injection showed left side aortic arch, no coarctation, no aortic incompetence, no PDA.
Assessment:
A 17 years old female adolescent with, D-TGA, ASD II with reactive pulmonary hypertension, on home oxygen and Sildenafil.
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WHAT TO DO ? SUGGESTIONS ?
Plan:
In view of the above-mentioned data, we think that this patient can go for D-TGA repair either arterial switch (after preparation of left ventricle) and ASD closure, leaving small hole or atrial switch. Patient still on home oxygen, Sildenafil 25 mg b.i.d., Aspirin tablet 81 mg once daily.
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On 31st May 2008 / 26-5-1429 The pt. presented to us after card.cath.followed by Atrial Switch at KFSH-Jed. . The patient was happy, she can move around and take care of her self.
Examination was:
Pulse: 83 b/min, BP 124/69 (86)mmHg, O2 saturation 93% in R.A. The pt. was adviced to continue on same medications and to follow w us when ever she come back from YEMEN .
13th August 2012 / 25-9-1433
Now she is 20 years old with a complain of palpitation with exercise intolerance and easy fatigability.
Medication:
Lasix 20 mg once daily, Digoxin 125 mcg once daily, Sildenafil 25 mg BID, Aspirin 81 mg once daily.
Examination was:
Pulse: 84 b/min, BP 107/77 (87)mmHg, O2 96% in R.A. S1+S2+O, soft systolic murmur grade 2/6. A 6 minutes walking test 300 meter with: O2 95% on R.A.
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ECG:
Sinus rhythm, short PR interval.
Holter Monitor:
Ectopic atrial tachycardia, minimum heart rate was 55 with sinus bradycardia and escape junctional rhythm, infrequent PVC.
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Repeated echocardiography findings: showed mild stenosis in baffle from left atrium to right ventricle with a maximum pressure gradient of 12 mmHg, no stenosis in baffle from right atrium to left ventricle, trivial mitral regurgitation with a pressure gradient of 42 mmHg, mild tricuspid regurgitation, no RVOT & LVOT obstruction, dilated main pulmonary artery and peripheral artery branches, fair myocardial function ( LVIDd 2.98 cm, LVIDs 2.25 cm, SF 24.5%, EF 50.3%).
Assessment:
A 20 years old female adolescent with D-TGA, S/p Atrial switch and palpitation with stenosis in the atrial buffle and atrial arrhythmia.
Plan:
So the patient was referred to Prince Sultan Cardiac Center for baffle stenting and Electrophysiologal Study .
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On October 2012, she presented to Prince Sultan Cardiac Center where she had astent in the atrial baffle.
Echocardiography showed stent was seen in systemic
venous baffle without obstruction showed a gradient of 7 mmHg, a dilated MPA, trivial PR, trivial MR, trivial TR was observed with good biventricular function.
EP Study:
1) junctional rhtyhm at the rate of 60 beats/min and recovered to sinus rhythm. 2) IART confirmed. 3) A ablation was done for delivering RF energy. By the end of the line completion, tachycardia was slow down and terminated upon reaching IVC; it was induced again and terminated by closing the gap in the line.
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However there was transient AV block that was not noticed due to junctional rhythm with last radiofrequency ablation. Successful intra atrial reentry tachycardia ablation.
ECG:
Sinus rhythm with HR 80/min.
Plan: Patient discharge on Aspirin.
On 27th October 2013, she is 21 years old, with shortness of breath and easy fatigability.
case
Examination:
She was looking, pink, & thin, Pulse 96 beats/min, BP 130/84 (99) mmHg, saturation 97 % in R.A., Wt 39.7 kg. Heart: S1+S2+ soft ejection systolic murmur grade 2/6. Chest: clear. Abdomen: clear.
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Repeated echocardiography findings: showed good myocardial function, mild MR, LV pressure more than 70 mmHg, stent is seen in place in the atrial tunnel.
Assessment:
D-TGA, S/P arterial switch with pulmonary hypertension. Plan: Continue on Aspirin 81 mg P.O OD, Lasix 20 mg P.O BID, Concor 5 mg P.O OD, Aldactone 25 mg P.O OD and Sildenafil 25 mg P.O B.I.D.
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the patient was still complaining of shortness of breath and easy fatiguability. Clinical examination: showed oxygen saturation 99 % in R.A with stable vital signs. Chest was clear, no organomegally. On 29th December 2013 / 26-2-1435
Echocardiography: LV pressure is 90 mmHg, with good function. The patient travelled back home (Yemen) on:
Aspirin 81 mg P.O OD, Lasix 20 mg P.O BID, Concor 5 mg P.O OD, Aldactone 25 mg P.O OD Sildenafil 25 mg P.O T.I.D.
DISCUSSION: 1. HAVE WE DONE GOOD TO THE PATIENT? 2. DIFFICULTIES TO PROVIDE THE REQUIRED MEDICATIONS FOR PULMONARY HYPERTENSION IN HER COUNTRY. 3. IS IT WISE TO LEAVE SUCH PATIENT ALONE WITHOUT INTERVENTION? 4.
IART , VENT.ECTOPICS , JNCTIONAL TACHYC.
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ACKNOWLEDGEMENT 1. Dra. Manal Al Hebshi 2. Tatabai Midtimbang
THANK YOU tha@cscc2015
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