A rare coronary angiogram

 

A 35-year-old woman was admitted with atypical chest pain. Her electrocardiogram was normal. There was no rise in serum troponin level. Echocardiogram showed a structurally normal heart with preserved left ventricular systolic function. Her serum cholesterol was very high (total cholesterol of 7.7 mmol/L with LDL of 5.3 mmol/L) but there was no other conventional risk factor for coronary artery disease. In view of ongoing chest pain and high cholesterol, a coronary angiogram was done to rule out coronary artery disease (Figures 1 and 2; Supplementary material online, Videos S1 and S2). Answer: 3. Anomalous origin of left coronary artery from right sinus of Valsalva with retro-aortic course. Explanation Origin of the left main coronary artery (LMCA) from the right sinus of Valsalva is a rare congenital anomaly with an estimated incidence of 0.04%. It has been associated with sudden cardiac death in young persons. Identification of the course of the LMCA after its origin is important to stratify the risk of sudden death. A computed tomography coronary angiogram (CTCA) will identify the course of the LMCA after its origin but in 1985 Ishikawa et al. 1 described a method to determine the course of the LMCA by conventional invasive Figure 2 Left anterior oblique view. Figure 1 Right anterior oblique view.

European Heart Journal. Acute Cardiovascular Care, Volume 11, Issue 5, May 2022, Pages e2–e3, https://doi.org/10.1093/ehjacc/zuac030

Case of the month 2

 

A 71-year-old woman presented to the emergency department (ED) with weakness, dizziness and some episodes of cardiac sounding chest pain for few days. There was no history of diabetes or hypertension and she was a non-smoker.

She saw a cardiologist for dizziness about a year ago. Her ECG showed frequent ventricular ectopics and echocardiogram showed structurally normal heart with preserved LV function and hence she was reassured cardiac wise at that stage. Her dizziness was subsequently thought to be vertigo.

This time she thought her dizziness to be exacerbation of her usual vertigo but episodes of chest pain made her seek medical advice. She was known to have ulcerative colitis and was on mesalazine. On presentation to ED , her hs troponin was 541 (normal < 14). ECG showed  T wave inversion in V1-V4.

ECG-

 

In view of chest pain, increased troponin and ECG changes she was treated as acute coronary syndrome (NSTEMI) and had a coronary angiogram done which showed normal coronary arteries. An echocardiogram showed dyskinesia of the mid and apical part of the LV ventricle with hypercontractile basal segment.

This echo feature is typical of something called Takotsubo cardiomyopathy (because it resembles an octopus pot) or apical ballooning syndrome. See video and still images

This condition was first described in 1990.This happens predominantly in post-menopausal women after severe mental or physical stress and hence is also called broken heart syndrome. There was no obvious  trigger in our case. In an international registry of Takotsubo cardiomyopathy patients of 1758 patients 28.5% had no preceding trigger. (1)

  • Modified Mayo clinic diagnostic criteria for Takotsubo cardiomyopathy. Patients are assigned this diagnosis when they satisfy all criteria
    (1) Transient hypokinesis, akinesis, or dyskinesis in the left ventricular mid segments with or without apical involvement; regional wall motion abnormalities that extend beyond a single epicardial vascular
    distribution; and frequently, but not always, a stressful trigger
    (2) Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture
    (3) New ECG abnormalities (ST-segment elevation and/or T-wave inversion) or modest elevation in cardiac troponin
    (4) Absence of pheochromocytoma and myocarditis.
  • The prognosis is usually favourable and the LV function improves in 4-8 weeks’ time. Complications can happen in up to 20% patients and can include shock, thrombus formation, heart failure, cerebrovascular
    accident, ventricular tachycardia, left ventricular rupture and/or ventricular septal rupture. In our patient an echocardiogram done after 3 weeks showed LV function had almost returned to normal
    (see video below)
  • Treatment is usually supportive (treatment of heart failure and arrhythmia and anticoagulation if LV thrombus develops)
  • There is a small chance of recurrence

The first video shows echocardiogram on this presentation
The second video is echo after 3 weeks
Compare the contraction of mid and apical part of LV in 2 videos

in diastole

in systole

Echo after 3 weeks

Reference:
1. Templin C, Ghadri JR, Dikeman J et al. Clinical features and outcomes of Takotsubo (stress) cardiomyopathy. NEJM 2015;373(10):929-38.
2. Yoshihiro J. Akashi, David S. Goldstein, Giuseppe Barbaro and Takashi Ueyama Takotsubo Cardiomyopathy : A New Form of Acute, Reversible Heart Failure. Circulation. December 16/23, 2008,Volume 118, Issue 25

Case of the month 1

A 49-yr-old woman presented to her general practitioner (GP) with shortness of breath on exertion and mild ankle swelling for few weeks. She was a smoker and initial impression was that she probably suffered from smoking induced chronic obstructive pulmonary disease. Her BNP was slightly raised  at 156 pg/ml and hence her GP requested for an open access echocardiogram (TTE) which showed a mass in left atrium. A transoesophageal echocardiogram (TOE) was arranged for further evaluation and it showed the following-

As you can see in transoesophageal echocardiogram, there was a mass in left atrium attached to interatrial septum with a stalk and projecting into the left ventricle through the mitral valve causing obstruction in mitral inflow in diastole. This echo appearance is typical of left atrial myxoma.

  • Myxoma is the most common primary cardiac tumour and accounts for 30-50% of all primary tumours pf the heart. Myxomas are usually single and occur in the left atrium in 75% cases where they most commonly arise from the area of fossa ovalis. Rarely myxoma can be a part of Carney Syndrome (Autosomal dominant, multiple myxoma formation in cardiac and exrtracardiac tissue, spotty skin pigmentation, endocrine hyperactivity, other tumours like testicular tumour and pituitary adenoma). On echocardiogram myxomas can appear smooth surfaced but is more commonly irregular. They are typically nonhomogeneous in texture with lucent centres or areas of calcification.
  • Annual incidence is around 0.5 per million population (1)
  • Majority of the patients present with one or more of the classic triad of obstructive, embolic or constitutional features

Obstructive symptoms and signs–dizziness, sob, cough, pulmonary oedema and heart failure due to obstruction to mitral inflow by the tumour

Embolic manifestations — due to tumour embolism to systemic or pulmonary circulation depending on the location of the tumour

Constitutional features– fever, weight loss, fatigue, myalgia, arthralgia, muscle weakness, Raynaud’s syndrome. They are believed to be due to IL-6 released by myxoma tumour cells (2).

  • Transthoracic /trans-oesophageal echo is diagnostic in typical cases. TOE is more sensitive and specific compared to TTE. CT and Cardiac MR are helpful in case of small tumours and in cases with atypical appearances
  • Treatment is surgical removal as early as possible. There is about 3% chance of recurrence in sporadic cases though a more recent report found no recurrence after a mean follow up 72 months after surgical excision in 82 cases of LA myxoma (3). Chance of recurrence is higher in familial cases (20%)

References–

  1. MacGowan SW, Sidhu P, Aherne T, Luke D, Wood AE, Neligan    MC, McGovern E. Atrial myxoma : national incidence, diagnosis and surgical management. Ir J Med Sci. 1993 Jun;162(6):223-6.doi: 10.1007/BF02945200
  2. Mendoza CE, Rosado MF, Bernal L. The role of interleukin-6 in cases of cardiac myxoma. Clinical features, immunologic abnormalities, and a possible role in recurrence. Tex Heart Inst J. 2001. 28(1):3-7.
  3.  Vroomen M, Houthuizen P, Khamooshian A, Soliman Hamad MA, van Straten AH. Long-term follow-up of 82 patients after surgical excision of atrial myxomas. Interact Cardiovasc Thorac Surg. 2015 Aug. 21 (2):183-8.