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Feb 2024 DOI 10.14302/issn.2329-9487.jhc-24-4910
Ebstein’s anomaly is a rare congenital heart disease. It is usually associated with other congenital defects, particularly atrial septal defect/patent foramen ovale (PFO). We report here the case of Ebstein's disease revealed in a 23-year-old adult admitted for palpitations and exertional dyspnoea. He was in heart failure. The EKG showed atrial flutter . Cardiomegaly was present with a cardiothoracic index of 0.7. On echocardiography, the apical displacement of the tricuspid septal leaflet was 15 mm/m2 , the Celermajer index was Grade 3. There was a large thrombus floating in the right atrium. While awaiting surgical management, the patient was being treated with diuretics, digitalis and anticoagulants.
Apr 2023 DOI 10.14302/issn.2372-6601.jhor-23-4530
Objective Drawing up a balance sheet of 16 years follow-up of the sole case of Diamond-Blackfan anemia diagnosed in Togo with arguments of molecular biology. Observation T.S a boy, born on 5th september 2006 has been followed up since he was three months, for Diamond-Blackfan anemia (DBA) in whom there has been found the mutation of ribosomial protein RPS19 in july 2010. It was the first observation in Subsaharian Africa. The treatment by transfusions from december 2006 to december 2022 has been associated with iron chelation through deferoxamin and promptly with corticotherapy at the dosage of 2mg/kg/day. The corticotherapy has been reduced as a consequence of corticoresistance from the fourth week, then definitely interrupted after four months. The evolution is marked by a clinical improvement with a staturo-balanced curve, and during the last control of 28th december 2022, the child was 53 kg heavy and 160 cm tall. The monthly physical tests did not reveal any signs of eventual overloading and the echocardiography of 26th december 2022 was normal. On the biological plan, the rate of the haemoglobin had been stable around 50g/l as a resultant of a transfusion each 4 to 6 weeks of red blood cell pellet. The chelation of iron had been done through deferoxamin with a monthly control of serum ferritin. That serum ferritin was 738,39ng/mg at diagnosis before the beginning of transfusions and during the follow-up, we noticed an average of 2977,3ng/ml (range 1817,1ng/ml and 4448,5ng/ml) Conclusion Thanks to the regular transfusions derived from the survey of the parameters of iron and the use of deferoxamin, we have succeded in keeping alive during sixteen years a patient who caught a disease whose evolution is unpredictable.
Mar 2019 DOI 10.14302/issn.2641-5518.jcci-19-2692
Percutaneous device closure of atrial septal defects (ASD) has proven to be safe and it is nowadays the standard treatment for ASDs. Immediate or late device embolization is a rare but potential complication of every attempted ASD device closure. We report a case of asymptomatic Amplatzer Septal Occluder into the left ventricular outflow tract (LVOT) detected by routine transthoracic echocardiography 3 months after successful implantation in a stable patient.
Aug 2018
Background: Co-morbidities are associated with increasing risk of mortality, hospitalizations and costs of treatment in Chronic Obstructive Pulmonary Disease patients. Identification of Co- morbidities related to COPD phenotypes may guide individualized therapies and achieve better prognosis. Methods: A prospective study of one hundred ten patients of confirmed COPD diagnosis were carried out and divided into five different phenotypes with related co-morbidities. History taking, clinical examination, Chest X-ray, Computed chest Tomography, laboratory investigations, arterial blood gas, Echocardiography and Electrocardiography were done for all patients. St. George’s Respiratory Questionnaire, COPD assessment test (CAT score) and BODEx (BMI, FEV1, dyspnea and exacerbations) were used for assessment of disease impact on quality of life, severity, and exacerbation respectively. Results: Emphysema group were 31% among all cases with mean age 61.8±9.1, frequent exacerbator group and Chronic bronchitis phenotype were 18% with mean age 64.4±11.3, and 48.8±9 respectively. COPD with bronchiectasis group were 19% with mean age 60.3±6 and Asthma COPD Overlap Syndrome (ACOS) were 12% with mean age 62.8±15.8. There was significant difference as regards age between different group of phenotypes P- value <0.001. There was significance difference in BODEx index and in (CAT) score among different COPD phenotypes P-value 0.020, 0.001 respectively. There was significant difference in all items of SGRQ among different COPD phenotypes P–value 0.001. Diabetes was commonly presented in 50 % ACOS cases, Ischemic heart disease was present more in Emphysema 22.9%, Osteoporosis was more in COPD with bronchiectasis 28.6%, Cor-pulmonale was more present in frequent excerbator 65%, and Anemia more common in COPD with bronchiectasis 23.8%. Depression was more common in frequent excerbator phenotype (45.0 %). Gastro-esophageal reflux was the most common co-morbidities (58 %) then cor-pulmonale 41.8%, systemic hypertension 40 % and pulmonary hypertension 28%. Conclusion: The presence of significant co-morbidities is important modifying risk factors for severity in COPD. They contribute to the overall severity in individual patients, have a major impact on quality of life, and major causes of hospitalization. Co-morbidities can be associated with any clinical phenotype.
Jun 2018 DOI 10.14302/issn.2372-6601.jhor-18-2139
Pulmonary hypertension (PH) has become an increasingly recognized complication in sickle cell anaemia (SCA) and is a major cause of morbidity and mortality. Though the burden of SCA in sub-Saharan Africa is high, there is paucity of data on SCA-associated PH with little or no attention given to it in routine patient care. The current study therefore sought to determine the prevalence of PH and its associated risk factors among adult patients with SCA. This was a cross-sectional study involving 76 clinically stable, hydroxyurea-naive participants. We obtained socio-demographic and clinical history. Measurement of Tricuspid Regurgitant jet Velocity (TRV) was obtained via transthoracic echocardiography and lung function was assessed using spirometry and pulse oximetry. Other investigations were complete blood counts, free plasma haemoglobin, serum urea and creatinine. Twenty-five (32.9%) of study participants had elevated TRV (≥ 2.5m/s) on Doppler echocardiography, which was suggestive of raised pulmonary artery systolic pressure. There were significant associations between elevated TRV and steady-state haemoglobin (p < 0.001), blood urea level (p = 0.030), presence of chronic leg ulcers (p = 0.043) and oxygen saturation (p < 0.001) and these may be identifiable and modifiable risk factors for selective screening with echocardiography in a resource poor setting.
Sep 2017 DOI 10.14302/issn.2576-9359.jot-17-1594
Constrictive pericarditis (CP) represents a rare complication after heart transplantation (HTx), resulting from various postoperative events such as mediastinitis, pericardial effusion, or allograft rejection. We describe our recent experience with managing an HTx recipient who developed atypical patterns of CP predominantly involving the right ventricle. A 52-year-old male who had received heart transplantation 2.5 years before was admitted to our institution because of progressive symptoms of heart failure. The patient had experienced acute rejection twice post-HTx, both with International Society for Heart and Lung Transplantation grade 1R, undergoing an additional endomyocardial biopsy other than those performed during regular check-ups. On admission, echocardiography revealed paradoxical septal motion and a large cystic-like mass with a thick capsule in front of the right ventricle. Right heart catheterization revealed elevation of right atrial pressure, with severely reduced cardiac index. Magnetic resonance imaging revealed both seroma and a thick cystic-like capsule tightly adhered to the right ventricle. CP was suspected despite the atypical patterns of presentation. Seroma was removed through exploratory lateral thoracotomy, without improvement in symptoms, which was only achieved via subsequent pericardiectomy involving resection of the thickened parietal pericardium, removal of effusion fluid, and further excision of diffusely thickened visceral pericardium and epicardium. The patient is currently recovering uneventfully. The possibility of CP after HTx should be considered despite the rarity of this condition and HTx recipients should be closely monitored using various imaging modalities because CP typically demonstrates non-specific symptoms and physical findings of heart failure, with high mortality.
Nov 2013 DOI 10.14302/issn.2329-9487.jhc-12-155
We report the case of a 22 year-old-male patient affected by Sotos syndrome was preoperatively and urgently evaluated for tracheal stent implantation due to respiratory insufficiency caused by idiopathic tracheal stenosis. Rest electrocardiogram detected a ventricular pre excitation; a transthoracic echocardiography showed a classic pattern fulfilling criteria for isolated left ventricular non compaction. At the best of our knowledge this is the first report of a patient suffering from Sotos syndrome and simultaneously affected by isolated left ventricular non compaction and ventricular pre excitation.
Nov 2013 DOI 10.14302/issn.2329-9487.jhc-13-272
Background: Conventional transthoracic echocardiography (TTE) and tissue Doppler imaging (TDI) are usually unable to reveal very early subtle abnormalities in left ventricular (LV) systolic function caused by hypertension, prior to manifestation of hypertrophy (LVH). This study was undertaken to assess whether speckle tracking echocardiography (STE) provides more insight into early hypertension induced LV systolic dysfunction, with the purpose of identifying patients at higher risk for heart failure (HF). Methods: Fifty patients (54.16 ± 8.4 years) and 50 controls (52.17 ± 8.6 years) were enrolled. According to the presence or absence of LVH, patients were classified as LVH(+) and LVH(–), respectively. Global longitudinal function was calculated by TDI, global strains (longitudinal (LS), radial (RS), and circumferential (CS)) were assessed by STE. Results: Conventional TTE showed a normal systolic function in all patients. TDI was able to detect a systolic dysfunction only in the LVH(+) group (P < 0.001) whereas STE revealed an impairment of systolic LS and CS in all patients, including those without hypertrophy (P =<0.0001). Furthermore, in the LVH(+) group, STE showed increased RS . Conclusion: In hypertensive patients, STE provides more detailed information than conventional echocardiography and TDI, since it reveals a systolic dysfunction before hypertrophy occurs (Stage A of ACC/AHA classification of HF) and identifies some early LV mechanic changes that might improve the clinical management of these patients
Mar 2013 DOI 10.14302/issn.2329-9487.jhc-12-153
We describe a rare case of multiple coronary artery-left ventricular fistulas associated with apical hypertrophic cardiomyopathy in a 62 year asymptomatic old male admitted to our department for a perioperative evaluation of non cardiac surgery, already diagnosed for multiple coronary artery-left fistulae. He underwent transthoracic echocardiography and then to accelerated dipiridamole stress-echo.