Cardiac Amyloidosis
Description
● Patient Profile
Age/Sex: 77-year-old man
➣ Chief Complaints:
The main complaints are dyspnoea and chest discomfort when exerting oneself.
➣Initial Diagnosis (3 years earlier):
• ST depression on the workout ECG suggests coronary artery disease (CAD).
• A negative ergonovine test (which ruled out vasospasm) and normal coronary angiography
• LV function was preserved on the echocardiography.
• Identified as having microvascular angina, or Cardiac Syndrome X, and receiving medical
• treatment
➣ Current Admission Findings
➣Symptoms: Progressive dyspnea, chest pain, cardiogenic shock
➣ECG:
• Limb leads shows Low voltage
• Precordial leads shows poor R-wave progression in
➣Chest X-ray:
• Cardiomegaly
• Interstitial congestion
• Bilateral pleural effusion
➣ Laboratory & Imaging
• Troponin-I: slightly higher than normal (0.15 ng/mL; < 0.1).
• BNP: Noticeably higher than normal (5643 pg/mL; normal < 100).
• Liver/Renal Function: Normal
➣Echocardiogram:
• Little thickening of the free and septal walls
• LV enlargement and worldwide hypokinesia
• Significantly lower EF (~29%)
• Pattern of restrictive filling (E/A ratio = 2.3)
• Low velocity of tissue Doppler E′ (2 cm/s)
• Coronary angiography repeat: No discernible stenosis
➣Risk of Misdiagnosis
• Due to early presentation (normal coronaries, usual angina), Cardiac Syndrome X was
misdiagnosed.
• Nevertheless, cardiac amyloidosis was already evident in the ECG and echocardiography.
ECG Indications
More than half of cases with amyloidosis have low voltage in the limb leads and poor R-wave progression.
Typical signs of cardiac amyloidosis include:
Low voltage (46 percent).
Pattern of pseudo-infarct (47%).
Both combined (25%)
Increased wall thickness combined with low voltage makes cardiac amyloidosis unique.
For instance, according to Rahman et al., septal thickness more than 1.98 cm plus low voltage
equals 72% sensitivity and 91% specificity.
Indicators of Echocardiography
Wall thickening, which is caused by amyloid infiltration, may resemble hypertrophy.
pattern of restrictive filling with a high E/A ratio >2
Even with little thickening, tissue Doppler displays early diastolic dysfunction.
Late in the illness, systolic dysfunction manifests.
Protocol
Management :
The case study illustrates how the progressive and systemic character of cardiac amyloidosis
poses significant management and nursing concerns. With an emphasis on the issues faced and
how each was handled in this instance, the following provides a thorough and organised
summary of management and nursing actions.
Through a mix of cutting-edge medical treatments, device assistance, and eventually heart
transplantation, cardiac amyloidosis was controlled. Nursing interventions focused on symptom
management, monitoring, and rehabilitation.
Problems that Heart Patients Face
Progressive heart failure that doesn't respond to standard medical treatment
Coronary artery stenosis is not the cause of recurrent chest discomfort.
Delays in recognising amyloidosis due to diagnostic misunderstanding with Syndrome X
The progression of haemodynamic instability to cardiogenic shock
Significant diastolic dysfunction that is not responsive to standard heart failure treatments
Significant fluid overload and pleural effusion
Deteriorating heart function causes psychological discomfort and functional disability.
Medical Support
Initial Medical Management: Standard drugs were used to treat the patient's anginal symptoms
once they were diagnosed with Syndrome X.
Assessment and Re-evaluation: The diagnosis and treatment strategy were modified after repeat
echocardiograms, electrocardiograms, and laboratory analyses showed amyloidosis-typical
symptoms (restrictive physiology, low voltage ECG, lower ejection fraction, raised BNP).
Haemodynamic Support: An intra-aortic balloon pump (IABP) was utilised for three months to
increase cardiac output and stabilise the patient when medication treatment failed to control
heart failure.
Definitive Therapy:
A heart transplant was done because the patient's heart failure had gotten worse despite the best
medical care. Despite being the sole treatment for end-stage cardiac amyloidosis, this procedure
is still debatable because of the possibility of recurrence and related complications.
Monitoring and Device-Based Support
Haemodynamic Monitoring:
To assess heart function and fluid status, vital signs, urine output, and BNP/lab results are
continuously monitored.
Respiratory Support:
Monitoring for respiratory distress, administering more oxygen as needed, and using diuretics
to treat pleural effusions and pulmonary congestion.
Device Management:
The IABP was overseen by nursing professionals, who also handled infection control,
immobilisation, site care, and limb ischaemia and haemorrhage evaluation.
Post-Transplantation Care:
Close observation for infection, rejection of the transplant, and general heart function after
surgery. Nursing was essential for wound care, early problem diagnosis, and drug compliance
(immunosuppression).
Nursing Intervention
Management of Symptoms and Fluids
Monitor for Signs of Heart Failure:
Check for oedema, weight increase, jugular vein distention, chest discomfort, and dyspnoea on
a regular basis. For early action, promptly notify the medical staff of any changes.
Monitor Fluid Balance:
Keep track of your daily weight, intake, and output, and check for any pleural effusions that may
be present or getting worse. To treat pulmonary congestion and volume overload, take diuretics
as directed. If further diuretic medication or other treatments are required, work with your
doctors.
Discomfort management:
Give analgesics as directed for chest discomfort. Keep an eye out for non-cardiac reasons and
assess pain symptoms for any changes that could call for more research.
Device and Medical Therapy Support
Care for Intra-Aortic Balloon Pump (IABP):
Observe stringent aseptic procedures when caring for the insertion site.
Keep an eye on lower limb perfusion; measure temperature, skin colour, and pulses to identify
any early issues like infection or ischaemia.
To avoid displacement and guarantee the effectiveness of the gadget, immobilise the afflicted
leg.
Vital Signs and Hemodynamic Monitoring:
To guarantee early detection of deterioration, regularly assess blood pressure, heart rate,
oxygen saturation, and perfusion status, particularly during cardiogenic shock or device usage.
Post-Transplant Interventions
Monitor for Rejection and Infection:
Keep a close eye out for symptoms of graft malfunction, fever, arrhythmias, or hypotension.
Always follow stringent aseptic measures. As directed, provide immunosuppressive and
preventative drugs, and provide information on infection prevention and adverse effects.
Supportive Rehabilitation:
After surgery, promote progressive mobility, assign patients to physical therapy, and keep an
eye out for symptoms of exhaustion or overexertion. Care plans should be modified to
accommodate each person's functional ability and tolerance.
Psychosocial and Educational Care
Patient and Family Education:
Inform patients about the course of the disease, their treatment plan, symptoms of heart failure
getting worse, how to take care of their devices (if any), and how to change their lifestyle to
recover. Give vocal and written advice on how to get medical help if you have any concerning
symptoms.
Psychological Support:
Provide emotional support, promote communication on diagnosis and prognosis, and arrange
for counselling or psychiatric treatment if necessary.
Notes
For More Information visit https://my.clevelandclinic.org/health/diseases/22598-cardiac-amyloidosis
