An 87-year-old female presented to the emergency department (ED) complaining of chest PAIN (CP). Her PMH includes hypothyroidism and rosacea. While shopping, patient developed sudden onset crushing central CP that prevented her from ambulating. Initially, she attributed the pain to her hiatal hernia. However, the pain persisted with radiation to the jaw and upper back.
In the ED, patient complained of constant CP described as a “pressure” like sensation. She denied having similar symptoms in the past. There was no shortness of breath, diaphoresis or nausea. At baseline patient was noted to have good exercise capacity. She denied any family history of sudden cardiac death or ischemic heart disease.
Initial Troponin I and CPK were normal. Her EKG showed no acute changes when compared to prior ones. Physical exam and vital signs were normal. Patient reported relief in symptoms after sublingual nitroglycerine.
EKG and cardiac enzymes (CE) were repeated after 6 hour. EKG remained unchanged. However, the CE were significant for Troponin I of 9.21, CPK 610 and CK MB 66.3. Patient’s chest pain and elevated CE were consistent with a diagnosis of Non STEMI and was taken for emergent coronary angiography. The study revealed hyperdynamic LV extending from mid to apical segments with akinesis of the apex. No LV outflow tract obstruction was observed. In addition long myocardial bridge was noted in the mid LAD with complete obliteration of the septals during systole.
Discussion:
In HCM, left ventricular hypertrophy usually develops in a dynamic pattern and can present during all phases of life, from infancy to old age. Various patterns of asymmetric LV hypertrophy have been reported.
AHCM is more frequently reported in the Asian population and has been observed in 41% and 15% of HCM patients in China and Japan respectively2. In contrast, the prevalence is only 2% in the United States. It is most commonly seen in males and the mean age is 41.4 years. An estimated 46% of patients are asymptomatic4. CP is described as the most reported symptom followed by palpitations, dyspnea and syncope5.
The overall prognosis is good. However, complications such as arrhythmias, myocardial infarctions, and embolization have been reported. Physical exam may reveal a soft systolic murmur with a forceful outward systolic thrust at the apex. The most common EKG finding is a negative T wave in pre-cordial leads seen in 93 % of the cases. T wave inversion greater than 10mm was observed in 43% of cases. The EKG in our case was atypical without the characteristic T wave inversions presenting a diagnostic challenge.
The best initial test is always a transthoracic echo (TTE), which shows LV apical hypertrophy. Contrast echocardiography can also be used to increase the diagnostic yield6. Other imaging includes contrast ventriculography which shows distinctive LV “spade-like” configuration of LV during diastole7. The most accurate test is a cardiac MRI. Treatment consists of beta blockers or calcium channel blockers for symptomatic relief.
Conclusions:
This was a new onset CP in an otherwise functional patient. Prior ECHO did not reveal apical HCM and her EKG did not show the T wave inversions. Cardiac MRI done outpatient revealed systolic obliteration of the mid/distal left ventricular cavity with apical sparing and no LV outflow tract obstruction. There is a need to increase awareness of this disease especially as it is likely underdiagnosed given its relatively benign course.