Case report on the radiographic features of epipericardial fat necrosis in a patient presenting with acute chest pain.

Epipericardial fat necrosis (EFN) is a rare and benign condition. The three cardinal features of EFN are acute pleuritic chest pain, radiological features of an encapsulated fatty lesion within the epipericardial fat and the presence of inflammation within the surrounding pericardium. The exact aetiology is still unknown, and there are no known risk factors. The condition appears to be a transient process with no associated long-term complications. In this case study, we demonstrate the multitude of radiological features associated with this condition. A 29-year-old Caucasian female presented with acute pleuritic chest pain. There were no acute changes on her blood work, electrocardiogram (ECG) or echocardiogram. The chest radiograph showed an opacity projected within the anterior mediastinum. Further imaging, with computed tomography (CT) chest with contrast, was performed to define the characteristics of the opacity. This confirmed an encapsulated, mixed fat, soft tissue density in the left pericardiac region deemed to represent EFN. Follow-up magnetic resonance (MR) cardiac imaging at 1, 4 and 12 months demonstrated the self-resolving characteristics of this condition. EFN is a rare differential diagnosis to consider in patients presenting with acute pleuritic chest pain when laboratory tests are normal and there are no acute ECG findings. EFN should be excluded by imaging with CT or MRI of the heart. We have demonstrated through follow-up MR imaging the progression and resolution of EFN over 12 months.

Fontan-associated nephropathy: Predictors and outcomes.

BACKGROUND: Nephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown. METHODS: The Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up. The Modification of Diet in Renal Disease (MDRD) equation was used to calculate eGFR. Patient outcomes were obtained from the Registry. Fontan failure was defined as death, transplantation, plastic bronchitis, protein losing enteropathy, Fontan takedown and NYHA class III-IV. RESULTS: Serum creatinine measurements were available for 328 patients. Renal dysfunction was defined as eGFR <90 mL/min/1.72m2. Renal dysfunction was present in 67/328 (20%) and 3/328 (1%) patients had an eGFR <60 mL/min/1.72m2. The 10-year survival and 10-year freedom from death and transplantation were the same, 96% (95% CI: 0.9-1) for those with renal dysfunction, and 89% (0.83-0.95; p = 0.1) and 87% (95% CI: 0.81-0.94; p = 0.05) for patients without dysfunction. The 10-year freedom from failure were also similar, 83% (95% CI: 0.70-0.97) for those without renal dysfunction vs 80% (95% CI: 0.74-0.89; p = 0.84). There was no change in mean eGFR for the renal dysfunction group over a mean of 8 ± 5.5 years. CONCLUSION: By the time they reach adulthood, 20% of patients with a Fontan circulation have renal dysfunction by eGFR calculation. Over the course of one decade, Fontan-associated nephropathy appears well tolerated.

Sequential Right and Left Ventricular Assessment in Posttetralogy of Fallot Patients with Significant Pulmonary Regurgitation.

BACKGROUND: The natural history of right ventricular (RV) and left ventricular (LV) size and function among adults with tetralogy of Fallot (TOF) repair and hemodynamically significant pulmonary regurgitation (PR) is not known. The main aim of this study was to determine changes in RV and LV size and function over time in an adult population with TOF repair and hemodynamically significant pulmonary regurgitation. METHODS: Forty patients with repaired TOF and hemodynamically significant PR were included. These patients were identified on the basis of having more than one CMR between January 2008 and 2015. Patients with a prosthetic pulmonary valve or any cardiac intervention between CMR studies were excluded. Rate of progression (ROP) of RV dilation was determined for both indexed right ventricular end-systolic volume (RVESVi) and indexed right ventricular end-diastolic volume (RVEDVi), and calculated as the difference between the last and first volumes divided by the number of years between CMR#1 and CMR#2. Subjects were also divided into two groups based on the distribution of the ROP of RV dilation: Group I-rapid ROP (>50th percentile) and Group II-slower ROP (≤50th percentile). RESULTS: The interval between CMR#1 and CMR#2 was 3.9 ± 1.7 years (range 1-8 years). We did find a significant change in RVEDVi and RVESVi over this time period, although the magnitude of change was small. Nine patients (23%) had a reduction in right ventricular ejection fraction (RVEF) by greater than 5%, 13 patients (33%) had an increase in RVEDVi by greater than 10 mL/m2 and seven patients (18%) had an increase in RVESVi by greater than 10 mL/m2 . Median ROP for RVEDVi was 1.8 (range -10.4 to 21.8) mL/(m2 year); RVESVi 1.1 (range -5.8 to 24.5) mL/(m2 year) and RVEF -0.5 (range -8 to 4)%/year. Patients with a rapid ROP had significantly larger RV volumes at the time of CMR#1 and lower RVEF as compared to the slow ROP group. There was no overall significant change in LVEDVi, LVESVi, or LVEF over this time period. CONCLUSIONS: We have demonstrated, in a small population of patients with hemodynamically significant PR, that there is a small increase in RV volumes and decrease in RVEF over a mean 4-year period. We believe it to be reasonable practice to perform CMR at least every 4 years in asymptomatic patients with repaired TOF and hemodynamically significant PR. We found that LV volumes and function remained stable during the study period, suggesting that significant progressive LV changes are less likely to occur over a shorter time period. Our results inform a safe standardized approach to monitoring adults with hemodynamically significant PR post TOF repair and assist in planning allocation of this expensive and limited resource.

The Feasibility and Clinical Utility of Microsphere Contrast-enhanced Transthoracic Echocardiography in Adult Congenital Heart Disease.

BACKGROUND: Transthoracic echocardiography (TTE) plays a key role in adult congenital heart disease (ACHD). However, a significant number of studies are nondiagnostic due to poor image quality. Enhancement of the blood pool-tissue interface with contrast-enhanced TTE (CE-TTE) can improve image quality in suboptimal studies. The aim of this analysis was to evaluate feasibility and clinical utility of CE-TTE in the assessment of patients with ACHD. METHODS: A retrospective analysis of all CE-TTE performed in ACHD patients at our institution from August 2007 to May 2014 was performed. Endocardial definition scores (EDS) for each segment in the right and left ventricles were graded pre- and postcontrast imaging, as 1 = good, 2 = suboptimal, 3 = not seen. The endocardial border definition score index (EBDSI) was also calculated pre- and postcontrast imaging. RESULTS: Twenty patients with ACHD had 24 CE. Summation data for all ventricular EDS for unenhanced TTE vs. CE-TTE imaging was: EDS 1 = 136 vs. 314, EDS 2 = 119 vs. 72, EDS 3 = 162 vs. 31, respectively. Wilcoxon matched-pairs rank-signed test showed a significant ranking difference (improvement) pre- and postcontrast for the combined ventricular data (P < .0001) and the individual left and right ventricular data (all P < .0001). The EBDSI for combined ventricular data using CE-TTE was significantly lower than for noncontrast imaging (1.23 ± 0.49 vs. 2.06 ± 0.62, P < .0001). There was one minor contrast adverse reaction. CONCLUSIONS: CE-TTE resulted in significantly improved right and left ventricular endocardial definition and improved EDBSI. CE-TTE should be viewed as an additional imaging technique that is available to help assess patients with ACHD, especially those with nondiagnostic images.

Routine angiography in survivors of out of hospital cardiac arrest with return of spontaneous circulation: a single site registry.

BACKGROUND: Coronary revascularization in resuscitated out of hospital cardiac arrest (OOHCA) patients has been associated with improved survival. METHODS: This was a retrospective review of patients with OOHCA between 01/07/2007 and 31/03/2009 surviving to hospital admission. Cardiac risk factors, demographics, treatment times, electrocardiogram (ECG), angiographic findings and in-hospital outcomes were recorded. RESULTS: Of the 78 patients, 63 underwent coronary angiography. Traditional cardiac risk factors were common in this group. Chest pain occurred in 33.3% pre-arrest, 59.0% were initially treated at a peripheral hospital, 83.3% had documented ventricular tachycardia or ventricular fibrillation, 55.1% had specific ECG changes, 65.4% had acute myocardial infarction (AMI) as the cause of OOHCA and the majority had multi-vessel disease. ST elevation strongly predicted AMI. The in-hospital survival was 67.9% with neurological deficit in 13.2% of survivors. The group of patients who had an angiogram were more likely to have AMI as a cause of cardiac arrest (71.4% vs 40.0%, p = 0.01) and more likely to have survived to discharge (74.6% vs 40.0%, p < 0.01). Poor outcome was associated with older age, cardiogenic shock, longer transfer times, diabetes, renal impairment and a long duration to return of spontaneous circulation. CONCLUSIONS: Acute myocardial infarction was the commonest cause of OOHCA and a high rate of survival to discharge was seen with a strategy of routine angiography and revascularization.

Hypertrophic osteoarthropathy in Eisenmenger syndrome.

Hypertrophic osteoarthropathy secondary to Eisenmenger syndrome can produce severe disabling pain. We report two cases treated by intravenous pamidronate infusion with good symptomatic relief. Such therapy can greatly improve the quality of life of end-stage cyanotic congenital heart patients.