Unicuspid aortic valve is a rare congenital abnormality with a prevalence of 0.02% in the adult population. Left ventricular noncompaction is another congenital anomaly with an incidence of 0.05%. Unicuspid aortic valve with severe aortic regurgitation and left ventricular noncompaction was found in a 15-year-old deaf male patient who presented with shortness of breath, orthopnoea and intermittent palpitations. JRCD 2019; 4 (4): 106–108.

Objective: Although hypertension is the leading cause of cardiovascular complications, time to develop cardiovascular complications among hypertensive patients has not been adequately investigated in Ethiopia. Therefore, the aim of this study was to assess time to develop cardiovascular complications and its predictors among adult hypertensive patients at the Ayder Comprehensive Specialized Hospital, Ethiopia. Result: A total of 578 hypertensive patients from the Cardiology Department were included in the study and followed for a total of 60 months (median 28 months). Half of the participants (290, 50.2%) were females. The median age of subjects was 54 years. Out of the 578 hypertensive patients who were analysed, 25.4% of them developed a cardiovascular complication. The incidence rate was 8.25 per 1000 persons per month. Significant predictors in the development of cardiovascular complications among hypertensive patients were age [AHR = 1.03 (95% CI=1.016, 1.046)], baseline cardiovascular complications [AHR=3.03 (95% CI=2.009, 4.870)], proteinuria [AHR=3.9 (95% CI=1.3, 11.68)], baseline systolic blood pressure [AHR =1.01 (95% CI=1.003, 2.012)], and baseline diastolic blood pres‐sure [AHR = 1.013 (95% CI=1.005, 2.021)]. JRCD 2019; 4 (4): 96-100.

Endothelin receptor antagonist (ERA) therapy‐induced increase in liver transaminases in patients with pulmonary arterial hypertension (PAH) is an adverse effect of bosentan which is not fully understood. A 62‐year‐old female with a 1.5‐year history of progressively worsening dyspnoea was diagnosed with idiopathic PAH (IPAH). After 2 months of PAH treatment with bosentan, levels of liver transaminases were elevated and autoimmune hepatitis was diagnosed. This clinical case demonstrates the challenge of diagnosing autoimmune hepatitis induced by ERA therapy in clinical practice. A thorough understanding of the pathogenesis and clinical presentation of autoimmune hepatitis, as well as the possibility to perform specific investigations with multiple serological markers are crucial for establishing the correct diagnosis. JRCD 2019; 4 (4): 101-105.

Pulmonary arterial hypertension is a rare disease of pulmonary circulation with poor prognosis if left untreated or diagnosed with delay. Active screening for the disease is not effective in the general population. However, in populations at increased risk for pulmonary arterial hypertension, all efforts should be taken to establish an early diagnosis. Here, we present 2 cases of late diagnosis. The first patient presented with a clear clinical picture, while the second case describes a patient in the at‐risk population who received treatment too late to obtain satisfactory results. We discuss the pitfalls which led to delayed diagnosis that finally resulted in treatment failure. JRCD 2019; 4 (3): 47-50.

Sinus of Valsalva aneurysm (SVA) is a rare congenital heart disease. A possible complication of SVA is rupture into a heart chamber, causing volume overload, which could lead to heart failure and the requirement for surgical correction. Transthoracic echocardiography is a readily available diagnostic tool for assessment, while other methods of cardiac imaging (cardiac CT and MRI) confirm the diagnosis and are necessary for preoperative assessment of the pathology. We present a very rare case of right sinus of Valsalva aneurysm dissecting the interventricular septum which ruptured into the right ventricle, causing volume overload of the right heart chambers and progressive heart failure. The diagnosis was established by transthoracic echocardiography and confirmed by ECG‐gated CT. Successful surgical correction resulted in regression of dilatation and dysfunction of heart chambers and to functional recovery of the patient. JRCD 2019; 4 (3): 51-54.

Amyloidosis is a rare disorder which can affect multiple organs, with cardiac amyloidosis (CA) being one of its main clinical manifestations. CA has 2 distinct subtypes, AL amyloidosis and transthyretin amyloidosis (ATTR), with different histopathological appearances and subsequently different treatment strategies. The diagnostic methods available include multiple modalities to properly detect CA, ranging from the surface electrocardiogram and simple 2D‐echocardiography to more sophisticated methods such as specific biomarkers, speckle tracking echocardiography, or even bone scintigraphy with radioisotopes. However, the definitive diagnosis is reached with an endomyocardial biopsy or a biopsy from peripheral tissue. CA is a particularly challenging disorder in terms of patient management, which ranges from symptomatic treatment for heart failure symptoms to special medication and multiple drug regimens. For AL amyloidosis, a 3‐drug regimen is used as a first‐line therapy including cyclophosphamide, a proteasome inhibitor, and dexamethasone. For ATTR amyloidosis, specific medications are used such as Tafamidis, depending on the specific stage of the disease. Clinical trials of new drugs are pending with the ultimate goal of improving treatment rates and quality of life in patients with any type of CA. JRCD 2019; 4 (2): 34–41