The East Timor Hearts Fund has provided cardiac services in Timor‐Leste since 2010, conducting three clinics yearly.
Timor‐Leste is one of the poorest countries in the world. The East Timor Hearts Fund is a charitable organisation involving Australian cardiologists providing outreach screening and access to cardiac interventions.
Background Lymphocytic myocarditis is an uncommon condition with a variety of clinical presentations. Isolated involvement of the right ventricle (RV) is very rare. We present a case of a young woman who developed right ventricular dysfunction and arrhythmias as a consequence of this condition, which appeared to be chronic at diagnosis. Case summary A 26-year-old lady was admitted to hospital following routine echocardiography, requested for screening of pulmonary hypertension in the context of known hypersensitivity pneumonitis. This echocardiogram demonstrated severe right ventricular dilatation and impairment. She was also experiencing atrial fibrillation and non-sustained, symptomatic episodes of ventricular tachycardia. Endomyocardial biopsy revealed lymphocytic myocarditis. She was managed with azathioprine and prednisone, as well as sotalol and apixaban for her atrial fibrillation, and has had no complications in the 12 months since discharge. Discussion Lymphocytic myocarditis isolated to the RV has only been reported in two previous cases, both of which were acute, dramatic presentations. This is the first report of a chronic example of this disease process. Due to her intercurrent immunosuppression, this patient may have been pre-disposed to the condition either by re-activation of a latent viral infection or partial treatment of a true autoimmune lymphocytic myocarditis.
BACKGROUND:The East Timor Hearts Fund (ETHF) is a charitable organisation of Australian cardiologists providing outreach screening in Timor-Leste. For patients requiring intervention, ETHF arranges logistics, procedures, and postoperative care. The aim of this project is to evaluate outcomes of patients requiring intervention.METHODS:The ETHF database of all patients was utilised to identify patients with disease warranting surgical or percutaneous intervention. Both patients who underwent intervention and those who did not proceed to intervention were included in this study. Patients who had intervention arranged by other organisations but have then had follow-up with ETHF were also included. Overall demographics and pre and postoperative factors were assessed, with sub-group analysis of adult and paediatric patients to identify any differences in care.RESULTS:Of 221 patients requiring intervention, 101 patients underwent intervention, receiving 22 different operations or procedures. Patients were predominantly young (median age 17.5 years) and female (64.7%), with rheumatic heart disease (63.8%). Twenty-four (24) (33.3%) women aged 15-45 years old with cardiac disease warranting intervention were documented as pregnant or breastfeeding at time of clinic assessment. Of patients who did not proceed to intervention, adults were more likely to be lost to follow-up (42.4% vs 18.5%) while paediatric patients were more likely to experience progression of disease (18.5% vs 7.5%, p=0.005). Median waitlist time was 5 months, with no significant difference between adults and children, correlating with a preoperative mortality rate of 5.4%. For patients who underwent intervention, post-procedure mortality was extremely low (0.9%) and attendance of at least one post-procedure review was excellent (99.0%). Eleven (11) (10.9%) patients have required repeat intervention, with no difference in rates between adult and paediatric patients. Length of follow-up extends up to 20 years for some patients.CONCLUSION:The Timor-Leste interventional cohort was predominantly a young female population with rheumatic and congenital cardiac disease. There were also high rates of pregnancy amongst female patients with severe cardiac disease. Delayed access to intervention may result in preoperative adverse events and mortality, and is a key target for improvement. Patients who undergo intervention have very low post-procedural mortality, good adherence to early medical follow-up and good long-term outcomes.
Acquired Factor X deficiency is a rare coagulation disorder, which predominantly presents with mucocutaneous bleeding, and is associated with systemic amyloid light-chain (AL) amyloidosis in the vast majority of cases. There are, however, rare case reports of its occurrence in the context of acute respiratory infection, various malignancies, and following exposure to certain medications. We report a case of an 82-year-old man who presented with a Non-ST segment elevation myocardial infarction, decompensated biventricular failure, Respiratory Syncytial Virus (RSV) pneumonia, active haemoptysis, and a profound coagulopathy. Initial laboratory studies were consistent with acquired isolated Factor X deficiency (prolonged Prothrombin Time and Activated Partial Thromboplastin Time, correction with mixing studies, factor X level < 3%), however Congo red staining for AL amyloidosis on abdominal fat biopsy was negative. The patient was acutely managed with prothrombinex therapy with partial but temporary reversal of their coagulopathy and resolution of their bleeding. Unfortunately, solid organ biopsy was deemed unsafe and the patient passed away from recurrent sepsis before a formal diagnosis of the underlying aetiology could be made. This article describes a diagnostic approach to coagulation factor deficiencies and AL amyloidosis, and their limitations in the coagulopathic patient. It further reviews the correlation between severity of bleeding complications and Factor X coagulant activity. Lastly, it outlines potential treatment options for acquired Factor X deficiency; such as coagulation factor replacement, plasma exchange, and targeted amyloidosis therapies.
Coronary CT angiography (CCTA) is a rapidly evolving technology which can characterise and image sub clinical atherosclerotic plaque and visualise anatomy and quantitate stenosis. Concern about radiation exposure has limited the uptake of this technology. The aim of this study was to review the radiation dose data in 2298 consecutive patients referred to a single centre in an Australian outpatient setting over 27 months using all available radiation dose reduction strategies. Prospective ECG gating ("step and shoot") was used preferentially in 2025 patients with a mean effective dose of 3.39 ± 1.84 mSv (range 0.86-12.6 mSv). For clinical reasons only 273 patients required retrospective ECG gating, mean dose 19.21 ± 5.58 mSv (range 2.4-34.9 mSv) resulting in an 85.7% reduction in dose for the majority of patients with the low dose technique. In conclusion, most patients referred for routine CCTA can be studied with a radiation dose comparable to invasive X-ray angiography and less than radionuclide myocardial perfusion imaging.
A 76-year-old woman presents with acute pulmonary oedema and cardiogenic shock 10h after elective electrical cardioversion for atrial fibrillation. Her echocardiogram shows new wall motion abnormalities with akinesis of the apical and mid segments of the left ventricle and her resting ECG contains deep T wave inversion and QTc prolongation. Angiography reveals non-occlusive coronary artery disease. The echocardiogram on day 6 shows resolution of left ventricular wall motion abnormalities and a return to normal systolic function. The diagnosis of tako-tsubo cardiomyopathy was made. This is the first report of this condition precipitated by electrical cardioversion.