INTERESTING CASE: ISCHEMIC

HEART FAILURE IN A PATIENT

WITH THALASSEMIA

INTERMEDIA AND PULMONARY

HYPERTENSION

Χρονόπουλος Παναγιώτης ,Ειδικευόμενος Καρδιολογίας ΠΓΝΠ

Chronopoulos Panagiotis, Resident Cardiologist ,University Hospital of Patras

History

▪ 38years old ,Female

▪ STEMI Anterolateral received thrombolysis in cardiogenic shock ,and recurrent episodes of VT referred to us for Rescue-PCI

▪ Thalassemia intermedia with repeated treatment with transfusions,1st transfusion and Splenectomy at the age of 5

▪ According to her medical records undisciplined to the follow-up and her iron overload treatment

▪ Smoking

▪ Established osteoporosis, hypothyroidism, liver iron overload and fibrosis

ECG

❑ Hospitalization due to

typical angina and

severe anemia Hb: 7

g/Dl

❑ Received transfusion

therapy

❑ Asymptomatic until

sudden crescendo

angina occurs

❑ First ECG associated

with the symptom

❑ Thrombolytic treatment

with tenecteplase was

administered

ECG early after thrombolytic treatment

30 min after

thrombolytic

treatment was

administered

Killip Class IV

▪ The patient developed cardiogenic shock and repeated episodes of VT terminated with DC shock and Lidocaine

▪ Vasopressors were administered to stabilize the patient

▪ Immediate transfer to our Centre for Rescue-PCI

ECG On arrival

Cardiogenic shock

and signs of overt

heart failure persist

Coronary Angiography Left System

LAD & LCX total occlusions. The culprit lesion is the Left Circumflex

RCA

LCx Primary PCI

PCI+Stent 2,75/12 Promus 2,75/12→ TIMI III Flow

LAD PCI

Wire Crossing successful but PTCA unsuccessful even with NC

Balloon→ TIMI 0 Flow

Final Result after Stent Implantation

PCI+Stent Promus 2,75/20 mm

Cath Lab & Cardiac ICU

▪ The patient during the Intervention was in cardiogenic shock with vasopressor support and in overt pulmonary oedema ( NIPPV-CPA – dilemma to further intubate and set to mechanical ventilation)

▪ The patient was transferred immediately after the intervention at Coronary-ICU where her status was gradually stabilized, weaned of the NIPPV and gradual decrease the dosage of vasopressors and good diuresis with iv loop diuretic

TTE Assessment

▪ Patient was stabilized and vasopressor treatment terminated

▪ Stable vital signs( BP:95/65mmhg and ventilation with a MV (FiO2: 35% )

▪ Evident signs of congestion with slow amelioration by intravenous loop diuretic

▪ ACEI , B-BLOCKERS intolerable because of hypotension

▪ The patient was transferred at the Echo Lab for assessment

TTE PLAX

Moderate LV Dilation, Reduced LVEF , Moderate MR, Trace Pericardial

Effusion

TTE PSAX

Anterior & Lateral Wall Severe Hypokinesia, Mild Dilation RVOT, Trileaflet

Aortic Valve with normal opening

TTE A4C

Depressed LVEF Mid-Apical IVS –Apical-Lateral Wall Severe Hypokinesia

Moderate Ischemic MR

TTE A2C & A3C

LV Hemodynamic Assessment: Diastolic Function

Grade II Diastolic Dysfunction

E=A ,E/e’=13,

Ischemic MR

EROA 0,33cm2

RV Function

RV Function (2)

Moderate-Severe TR,Tricuspid Annulus Mild Dilation, RV dilation, Severe Pulmonary Hypertension

PASP 70 mmhg, meanPAP ( PR Vel) : 40 mmhG ,PADP (PR Vel) : 15 mmhg

S’TDI normal IVRT> 35msec ( Sign of PHT ) , Dilated IVC < 50% Resp Change → High CVP

Speckle Tracking Echocardiography

Results-Discussion

▪ Depressed LVEF 35%

▪ Moderate Ischemic MR

▪ Grade II Diastolic Dysfunction, High

LV Filling Pressure

▪ Severe PHT

▪ Moderate Severe TR

▪ High CVP

Lack of Follow up

▪ The patients last cardiovascular imaging was an Cardiac

MRI 2008 normal LVEF 65% LvmassEDi= 51g/m2

▪ No signs of heart iron overload T2* relax time 40msec(

normal) ,R2*=25msec (normal)

▪ Last TTE 2004 ! No signs of PHT or structural heart

disease▪ . Wood JC, Enriquez C, Ghugre N, Tyzka JM, Carson S, Nelson MD, Coates TD. MRI R2 and R2*

mapping accurately estimates hepatic iron concentration in transfusion-dependent thalassemia and

sickle cell disease patients. Blood. 2005;106:1460 –1465

Clinical assessment

▪ The patients symptoms were refractory

▪ Due to hypotension intolerant to standard of care

medications (ACEI,B-BLOCKER)

▪ No weaning from mask ventilation and loop diuretics

▪ NYHA IV clinical status

▪ Need for extra iv diuresis in order to ameliorate

paroxysmal nocturnal dyspnea episodes and sleeping in

upright position

Our keys to diagnosis and PLAN

Ferritin Level: 2908 ng/ml

Lack of follow up , Established Iron Overload,

Undisciplined in iron overload treatment

High Output HF associated with thalassemia + Iron

Overload Cardiomyopathy with a devastating acute

decompensation due to massive infarction

Plan

▪ In close collaboration with the Hematology Department and the specific Thalassemia unit we decided to deliver the patient intravenous iron overload treatment with deferoxamine and check her status

▪ The patients clinical status was stabilized we managed to treat her with the lowest dose of bisoprolol( 1,25-2,5 mg) and ivabradine 5mg x2 , eplerenone 50mg

▪ After a period of stability a 2-day course with dobutamine (5 μγ/κg/min) improved the congestion and the patient weaned from 02 therapy and per os diuretics were given

▪ Discharge in a few days was our plan AND SCD assessment for ICD implantation in 40 days, and a CMR to assess iron overload

SCD beat us ALL

DC Defibrillation→

ROSC→Intubation→

Immediate Coronary

Angiography

Emergency Catheterization

LAD CTO , LCX stent patent, RCA patent ( No changes from baseline

angiography)

Outcome

▪ The patient was transferred at the cardiac ICU in cardiogenic shock refractory to very high doses of vasopressors

▪ Her status was complicated with resistant respiratory failure and anuria

▪ Died 2 days after the in-hospital cardiac arrest

First Case Reports

References

The American Journal of Medicine (2005) 118, 957-967

Blood. 2001 Jun 1;97(11):3411-6.

Iron Overload treatment AND Prognosis

THANK YOU VERY MUCH