Patterns of left ventricular hypertrophy and late gadolinium enhancement on cardiac MRI in patients with hypertrophic cardiomyopathy and their prognostic significance - An experience from a south Asian country

Objectives: Cardiac magnetic resonance (CMR) imaging is very pertinent in the diagnosis and risk stratification of patients with hypertrophic cardiomyopathy (HCM). We aimed to assess the patterns of left ventricular (LV) hypertrophy, late gadolinium enhancement (LGE), and their prognostic significance in HCM patients in Pakistani population, as no such data are available from Pakistan.Material and methods: This was a retrospective, single center study. All patients who had confirmed diagnosis of HCM on CMR at Aga Khan University Hospital during the period of 2011-2019 were identified and included in the study.Results: A total of 74 patients were included with the mean age of 45.6 ± 15 years and the majority 71.6 % (n = 53) being male. Maximal LV wall thickness was 21.1 ± 5 mm, asymmetrical septal hypertrophy being the most common pattern (62.2%, n = 46). LGE was present in 75.7% (n = 56) with most common site being septum plus LV free wall (24.3%, n =18). Mean ejection fraction% was found to be lower in patients with LGE (P \u3c 0.001). Major adverse cardiac events (MACE) were observed in 40.5% (n = 30). Presence of LGE and right ventricular involvement was found to have a statistically significant association with MACE (P value 0.018 and 0.046, respectively). In multivariable analysis, only LGE was significantly associated with MACE (odd ratio: 4.65; 95% CI: 1.21-17.88).Conclusion: Asymmetrical septal hypertrophy was the most common pattern of hypertrophy. LGE was present in three fourth of the study population and it was significantly associated with MACE

Cardiac magnetic resonance imaging in a young patient with left ventricular spongiform cardiomyopathy: A case report and review of literature

Left ventricular spongiform or non compaction cardiomyopathy is one of the rarer forms of cardiomyopathy with a reported prevalence of 0.014-0.05% in adults. It is characterized by severe left ventricular systolic dysfunction occurring secondary to the failure of the myocardium to fuse resulting in non-compacted myocardium with a trabeculated appearance of the myocardium. Initially described on the basis of 2-D echocardiography, the identification of left ventricular non compaction has now improved with the introduction of sophisticated imaging modalities such as cardiac magnetic resonance imaging that allows better visualization of the non-compacted myocardium assisting in accurate diagnosis. We report a case of isolated left ventricular spongiform or non-compaction cardiomyopathy that was diagnosed on cardiac magnetic resonance imaging with classical features. The patient was started on standard heart failure medications along with anticoagulation and continues to do well on follow up

Severe mixed mitral valve disease due to massive mitral annular calcification: A case report and literature review

We present an unusual case of massive mitral annular calcification (MAC) leading to severe mixed mitral valve disease, viz severe mitral regurgitation and severe mitral stenosis. To our knowledge, severe mixed mitral valve disease secondary to MAC is extremely rare. The patient (a 65-year-old lady) presented with worsening shortness of breath and signs of congestive heart failure. Echocardiographic examination revealed massive mitral annular calcification. Despite the massive annular calcification, she had experienced neither embolism nor endocarditis in the past. Because of severe symptomatic mitral regurgitation and mitral stenosis, surgery was advised; however, the patient declined it

Clinical presentation, cardiac magnetic resonance findings, and prognosis of patients with arrhythmogenic right ventricular cardiomyopathy - An experience from Pakistan

Objectives: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart-muscle disease, characterized by fibro-fatty replacement and ventricular arrhythmias, that primarily affects the right ventricle (RV). We aimed to look at the clinical presentation, cardiac magnetic resonance (CMR) imaging findings and prognosis of patients with ARVC in Pakistan.Material and methods: It is a retrospective observational study, 17 consecutive patients with CMR and other findings consistent with ARVC, were enrolled from 2010 to 2019 at a single center.Results: Out of 17 patients, 12 (70.6%) were male with a mean age of 33.5 ± 17.5 years. Family history of sudden cardiac death was present in 3 (17.7%) patients while one (5.9%) patient had family history of ARVC. Syncope was the first presenting symptom in eight (47.1%) patients. On 12 leads ECG, T wave inversion in precordial leads was found in 6 (35.4%) patients, and epsilon wave was present in only 3 (17.7%) patients. On echocardiogram, 13 (76.5%) patients had dilated RV with reduced systolic function. On CMR, majority of patients (n = 14, 82.4%) were found to have RV dilatation with regional dyskinesia and fatty infiltration, 9 (52.9%) of them had left ventricular involvement also. Follow-up was available for 14 patients (82.4%) with a mean follow-up period of 35.5 ± 19.7 months. Three (21.4%) of them died and 10 (71.4%) got admissions for heart failure during follow-up period.Conclusion: Arrhythmia related events are the main presenting symptoms of ARVC in this region, and left ventricular involvement in ARVC is not rare in this population. The mortality is relatively high, probably due to advanced disease at the time of presentation and less medical facilities available

Myocarditis mimicking acute coronary syndrome - the role of cardiac magnetic resonance imaging in the diagnosis

Myocarditis is an inflammatory disease of myocardium with a wide range of clinical presentations. Myocarditis may mimic acute coronary syndrome (ACS) and adequate differential diagnosis is not possible by conventional tests. Cardiac magnetic resonance (CMR) has emerged as a leading imaging modality in the diagnosis of myocarditis in such patients. Here, we report three cases of myocarditis mimicking ACS with normal coronary arteries. CMR was used for confirming the diagnosis of myocarditis in all three patients presented here

Large intra-cardiac fibroma identified on cardiac MRI - a case report and review of literature

Primary cardiac tumours are rare. Cardiac fibroma is a benign tumour of the heart. It is fairly common among children and adolescents and is rarely encountered in adults. We present the case of a thirty-eight year old lady who presented with shortness of breath and was found to have a very large intra-cardiac mass that had cardiac magnetic resonance (CMR) features consistent with cardiac fibroma. The patient was referred for tumour resection, however could not survive the surgery

Cardiac magnetic resonance evaluation of cardiac masses in patients with suspicion of cardiac masses on echo or computed tomography

Objectives: In recent years, cardiac magnetic resonance (CMR) imaging has emerged as an important tool in the identification and characterization of cardiac masses. No imaging data on cardiac masses are available from Pakistan. We aimed to review the clinical presentation, CMR findings, and outcome of patients referred for CMR due to suspicion of cardiac masses on echocardiogram or computed tomography (CT).Material and methods: We reviewed all the patients referred for CMR at Aga Khan University Hospital, Karachi, from January 2011 to March 2020, with the suspicion of cardiac mass on echocardiogram and/or CT. Only those with the confirmed diagnosis of cardiac mass on CMR were included in the study.Results: A total of 27 patients were referred for CMR from January 2011 to March 2020, with the suspicion of cardiac mass on echocardiogram and/or CT. Four patients were excluded as no cardiac mass was found on CMR. Out of 23 cases, majority (n = 15, 65%) were female, age ranging from 3 months to 70 years, with a mean age of 40 ± 22 years. Shortness of breath was the main presenting symptom (n = 19, 83%). Echocardiogram was the initial imaging modality done in all the patients while CT was also performed in 6 patients (26%). Out of 23 patients, 4 (17%) were diagnosed to have thrombus on CMR. In two cases, it was in the left ventricle with evidence of myocardial infarction on late gadolinium images. Myxoma was the most common tumor diagnosed on CMR in 6 patients (26%) followed by rhabdomyoma (n = 3, 13%) and fibroma (n = 2, 8.7%). There were three malignant primary tumors of the heart based on CMR appearances and one with tumor thrombus extension of hepatocellular carcinoma in the right atrium from inferior vena cava. Two patients were diagnosed to have non-neoplastic lesions - one with large intracardiac hydatid cyst and one with possible large fungal vegetation. Among 23 patients, 9 patients (39%) underwent surgery, 5 with myxoma, 2 with rhabdomyoma, 1 with fibroma, and 1 with fibroelastoma. Findings on surgery and histopathology matched the CMR diagnosis in all the patients except the one with the CMR diagnosis of myxoma in which histopathology was consistent with thrombus.Conclusion: CMR can play an important role in confirming the presence or absence of a mass in the heart. It can also provide differentiation of non-neoplastic and neoplastic lesions and among different types of neoplastic lesions with reasonable accuracy. However, the limitations of CMR must be recognized

Increased level of morning surge in blood pressure in normotensives: across-sectional study from Pakistan.

Objective: To determine the mean morning surge (MS) in blood pressure, the frequency of increased morning surge in normotensive subjects, and to compare those with morning surge with those without MS. Study Design: A cross-sectional, comparative study. Place and Duration of Study: The Department of Medicine, The Aga Khan University Hospital, Karachi, from April 2011 to March 2012. Methodology: Adult normotensive healthy volunteers aged 35 to 65 years were inducted. Their ambulatory blood pressure (ABP) was measured over a 24-hour period, using digital ambulatory blood pressure monitors. Morning surge was calculated as the average of four readings after waking minus the lowest three nocturnal readings. Increased morning surge was defined as \u3e 11 mm Hg in systolic (SBP) or \u3e 12 mm Hg in diastolic (DBP). Dipping was defined as \u3e 10% dipping in blood pressure. Results: Eighty-two healthy volunteers were recruited. Their mean age was 36.9 ±1.2 years; 74.4 (61%) were men, and 58.5 (48%) woke up for morning prayers. Mean overall SBP was 113 ±1.6 mm Hg, overall DBP was 73.9 ±0.7 mm Hg, and overall heart rate was 75 (10) beats/minute. Mean morning surge was 17.6 ±1.0 mm Hg in SBP and 16.0 ±0.8 mm Hg in DBP. The frequency of increased morning surge was 66 (80.5%) in SBP, and 57 (69%) in DBP. On comparison of participants with normal morning surge and increased morning surge in SBP, there was a significant difference in nondipping status (13.4% in normal vs. 18.3% in increased morning surge, p= 0.001). Conclusion: Mean morning surge in SBP and DBP are relatively higher in this subset population in a tertiary care center in Pakistan. These values are higher than those reported in the literature

Knowledge of coronary artery disease (CAD) risk factors and coronary intervention among university students

OBJECTIVE: To elucidate knowledge of coronary artery disease (CAD) risks factors and coronary intervention in adult students of Karachi East. To calculate the mean knowledge score about CAD risk factors among them. METHODS: A multi center crossectional study was conducted in Universities and colleges of Karachi East from April-September 2005. Questionnaires were distributed to 200 adult students of different non-medical universities and colleges. The questionnaire contained assessment of knowledge of risk factors on CAD and awareness about coronary angiography. Those belonging to medical colleges and universities were excluded from the study. Knowledge was assessed as a continuous variable. Risk factors for CAD were taken as categorical variables. RESULTS: The mean age of students was 20 yrs +/- 2.2 years and 62% were females. The mean score of knowledge about risk factors of CAD was 11.47 +/- 2.37. Sixty percent students thought that heart diseases are the number one cause of death in our population. Twenty five percent students graded smoking as the top most risk factor for CAD. Twenty five percent students refused to quit smoking for CAD prevention. Forty eight percent students correctly defined coronary angiography. Eighty five percent students thought that cost is the major hindrance in getting timely treatment. Knowledge of fifty percent students was based on personal and family experience of heart disease. CONCLUSION: Students graded smoking as the topmost risk factor for CAD and cost as the major hindrance in getting timely treatment for heart disease. Only half of the students were aware about coronary angiography. The mean knowledge score among them was above the median score but not up to the mark

Features on cardiac magnetic resonance imaging and accuracy of echocardiographic findings for diagnosing constrictive pericarditis

Constrictive pericarditis is a rare disease with a difficult diagnosis. Cardiac magnetic resonance (CMR) imaging data of Aga Khan University Hospital from January 2011 to March 2020 was retrospectively reviewed and patients with the diagnosis of constrictive pericarditis were included. A total of 22 patients were included with the mean age of 46±16 years and majority 17(77%) were male. The most common findings on transthoracic echo were significant respiratory variation in mitral and tricuspid inflow velocities in 20(91%), and septal annular e\u27\u3e9 in 10 (86%). The most common finding on CMR was respiratory septal shift in 22(100%), followed by septal bounce in 21(95%) and thickened pericardium in 18(82%). Nearly two-third of the patients, 15(70%) were considered for pericardiectomy but it was deferred in 5 patients due to high surgical risk. Ten patients underwent pericardiectomy, with no mortality on a mean follow up of 4±2 years