Unusually high creatine kinase in a case of rhabdomyolysis without acute kidney injury: a case report.

Background: While rhabdomyolysis frequently leads to hospital admissions, typically following trauma, recurrent occurrences are relatively rare, accounting for just 10% of cases. For young patients experiencing repetitive episodes without an apparent cause, a comprehensive investigation into the possible etiologies is crucial. Recognizing the atypical nature of recurrent rhabdomyolysis is crucial and a thorough workup encompassing evaluations for potential endocrine, inflammatory, and metabolic etiologies is recommended. Additionally, acute kidney injury is a common complication with severe rhabdomyolysis, hence early recognition and intervention is crucial. Case Description: Herein we present a case of a 30-year-old young African American male patient with recurrent rhabdomyolysis with the highest ever reported creatine kinase (CK) to our knowledge. A notable aspect of this case is the surprising absence of acute kidney injury, despite the severity of CK elevation. We also delve into the extensive workup done for rhabdomyolysis of unclear etiology. Conclusions: Our case underscores the importance of looking into non-traumatic factors behind recurrent rhabdomyolysis, especially in young patients. We also stress the significance of early detection and intervention, showcasing the potential to prevent acute kidney injury even in the presence of markedly elevated CK levels. Timely recognition and appropriate management can prove instrumental in mitigating the severity of complications associated with rhabdomyolysis.

Cystic bronchiectasis in sarcoidosis.

Pulmonary sarcoidosis can manifest in different radiologic patterns. Typical manifestations in high-resolution computed tomography are bilateral perihilar lymphadenopathy, micronodules, and fibrotic changes. Atypical manifestations are mass-like or alveolar opacities, honeycomb-like cysts, miliary opacities, tracheobronchial involvement, and pleural disease. Cystic bronchiectasis in pulmonary sarcoidosis is rare, with only a few reported cases in the literature. We present another case of cystic bronchiectasis with a honeycomb-like pattern in pulmonary sarcoidosis and with cardiac involvement. This case was presented as an abstract poster at the American Thoracic Society conference in 2022.

COVID-19 Bullous Lung Disease Superinfection by Raoultella planticola.

Bullous lung lesions from coronavirus disease 2019 (COVID-19) pneumonia, causing pneumothorax, are a rare complication, affecting up to 1% of infected patients. Raoultella planticola is an aerobic, gram-negative bacteria known to cause opportunistic infection. We present a rare case of spontaneous pneumothorax from rupture of lung bulla as a late sequela from COVID-19 pneumonia and superinfection of the bulla by R. planticola. Although superinfection of bullous lesions is known, this is the first reported case of R. planticola pneumonia in a patient with COVID-19 lung bullae. COVID-19 patients are at heightened risk for bullous lung lesions and superinfection by opportunistic organisms; thus, they should be followed up closely.

An Unusual Presentation of Thyrotoxicosis: Leg Weakness and Hypokalemia in a 21-Year-Old Male.

Patients with hyperthyroidism or thyrotoxicosis present with an unspecific constellation of signs or symptoms such as palpitations, tremors, weight loss, or diarrhea. In some severe cases, hyperthyroidism can predispose patients to metabolic abnormalities and arrhythmias. Thyrotoxic periodic paralysis (TTP) is a rare, life-threatening complication or variant of hyperthyroidism associated with hypokalemia and muscle weakness that affects young Asian or Hispanic males between 20-40 years. TTP is reversible, and the management consists of beta-blockers, antithyroid therapy, and conservative potassium correction to prevent severe cardiovascular events such as ventricular arrhythmias with the improvement of transient muscle paralysis. We present a case of a 21-year-old Hispanic male complaining with symptoms of thyrotoxicosis, marked hypokalemia, and severe generalized muscle weakness. Physicians must be aware of this uncommon complication of thyrotoxicosis called thyrotoxic periodic paralysis (TTP) to avoid potassium overcorrection and all the endocrine associations with this pathology.

Heroin-Induced Acute Pancreatitis.

Heroin-induced pancreatitis (HIP) is rare with only a few cases reported previously in the literature and the pathophysiology mechanism is yet to be investigated. We present two cases of acute pancreatitis (AP) in the setting of acute heroin (diacetylmorphine) intoxication. Both patients presented with nausea, vomiting and severe abdominal pain after intranasal heroin use. On laboratory analysis were found to have elevated serum lipase, positive urine toxicology for opioids, without any other obvious causes for AP. Both patients had a full recovery with supportive treatment. As a general approach, drug-induced pancreatitis is a diagnosis of exclusion and a high index of suspicion is required when the most common etiologies are ruled out.

A Rare Case of an Abdominal Aortic Thrombus Secondary to Syphilitic Aortitis.

Syphilis is a sexually transmitted spirochete infection whose presentation depends on the stage of infection. Currently, due to antibiotic treatment, tertiary syphilis is a rare clinical entity. When present, it is characterized by neurosyphilis, gummas, and cardiovascular infection. We present a case of a 64-year-old male who came with abdominal pain due to allergic colitis and was incidentally found to have a mural thrombus of his abdominal aorta. Following a negative workup and no etiologic cause of the thrombus, the patient was diagnosed with syphilitic aortitis. Previous cases have been seen in patients who present with infarction due to aortic thrombosis secondary to syphilitic aortitis. Practitioners must be aware that patients with tertiary syphilis, such as this patient, could have aortic thrombosis without any signs of ischemia and are at risk for infarction.

Left ventricular filling pressure in Tetralogy of Fallot: Correlation between invasive and noninvasive indices.

BACKGROUND: Left heart filling pressures, as measured by pulmonary artery wedge pressure (PAWP), is associated with heart failure related mortality. Because of the prognostic importance of PAWP, several echocardiographic indices have been proposed for noninvasive assessment of PAWP. However, these indices have not been validated in the congenital heart disease population. The purpose of this study was to determine the correlation between echocardiographic indices of PAWP, and the effect of high PAWP on transplant-free survival in adults with tetralogy of Fallot (TOF). METHODS: Retrospective study of adult TOF patients that underwent cardiac catheterization at Mayo Clinic, 1990-2017. We selected these pre-defined set of echocardiographic indices of LV diastolic function: mitral valve early velocity (E), mitral valve early and late velocity ratio (E/A), mitral valve deceleration time (DT), mitral annular tissue Doppler early velocity (e'), and left atrial volume index (LAVI). RESULTS: Of the echocardiographic indices analyzed among 213 patients (age 37 ± 14 years), only E velocity (ß = 5.83, standard error = 1.52, p < 0.001) and LAVI (ß = 0.14, standard error = 0.05, p = 0.007) correlated with PAWP. LAVI > 28 ml/m2 had sensitivity of 79% and specificity of 63% (AUC 0.712), and E velocity > 1.0 m/s had sensitivity of 66% and specificity of 89% (AUC 0.692), for detecting PAWP > 15 mmHg. LAVI > 28 ml/m2 was associated with reduced 10-year transplant-free survival (49% vs 90%, p < 0.001). CONCLUSION: This study supports the use of LAVI for noninvasive assessment of PAWP and for prognostication. Further studies are required to validate these results in a different population.

Incidence and outcomes of prosthetic valve endocarditis in adults with tetralogy of Fallot.

BACKGROUND: The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot. METHODS: Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990-2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis. RESULTS: A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis. CONCLUSIONS: The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.

The role of echocardiography for quantitative assessment of right ventricular size and function in adults with repaired tetralogy of Fallot.

BACKGROUND: Quantitative assessment of right ventricular (RV) systolic function by echocardiography is challenging in patients with congenital heart disease because of the complex geometry of the RV and the iatrogenic structural abnormalities resulting from prior cardiac surgeries. The purpose of this study was to determine the correlation between echocardiographic indices of RV systolic function and cardiac magnetic resonance imaging (CMRI) derived RV ejection fraction (RVEF) in adults with repaired tetralogy of Fallot (TOF). METHODS: Quantitative assessment of RV function was performed with RV tissue Doppler systolic velocity (RV s'), tricuspid annular plane systolic excursion (TAPSE), and fractional area change (FAC). These echocardiographic indices were compared to RVEF from CMRI performed on the same day as echocardiogram. RESULTS: Of 209 patients, the mean RV FAC was 39 ± 9%, TAPSE was 18 ± 4 mm, RV s' was 10 ± 2 cm/s, and RVEF was 40 ± 10%. There was a good correlation between TAPSE and RVEF (r = 0.79, P < .001), good correlation between RV s' and RVEF (r = 0.71, P < .001), and modest correlation between FAC and RVEF (r = 0.66, P < .001). TAPSE < 17 mm effectively discriminated between patients with RV systolic dysfunction defined as RVEF < 47% (sensitivity 81%, specificity 79%, area under the curve [AUC] 0.805). FAC < 40% was associated with RVEF < 47% (sensitivity 72%, specificity 63%, AUC 0.719). RV s' < 11 cm was associated with RVEF < 47% (sensitivity 83%, specificity 68%, AUC 0.798). CONCLUSION: Despite the structural and functional abnormalities of the RV in patients with repaired TOF, quantitative assessment of RV systolic function by echocardiography is feasible and had good correlation with CMRI-derived RVEF.

Coronary artery disease in adults with tetralogy of Fallot.

BACKGROUND: There are limited data about outcomes of coronary artery disease (CAD) in adults with repaired tetralogy of Fallot (TOF). The purpose of this study was to describe the prevalence and treatment of CAD in adults with TOF, and the impact of CAD on long-term survival. METHODS: Retrospective review of MACHD database for adults with repaired TOF who underwent aortic root/selective coronary angiogram, 1990-2017. Patients were categorized into three groups: (1) No CAD defined as normal coronary angiogram; (2) Mild CAD defined as ≤50% stenosis in all vessels; and, (3) Significant CAD defined as >50% stenosis in any vessel. RESULTS: We identified 105 (23%) of 465 TOF patients that had angiograms; mean age 47 ± 12 years. The prevalence of mild CAD and significant CAD was 19% (20 patients) and 15% (16 patients), respectively. Of these 16 patient with significant CAD, 9 (56%), 3 (19%), and 4 (24%) patients received guideline directed medical therapy, percutaneous coronary intervention, and coronary artery bypass grafting, respectively. Significant CAD was an independent risk factor for mortality (HR: 2.03, 95% CI 1.64-4.22, P = .022) after adjustment for differences in age, and prevalence of atrial fibrillation and renal dysfunction. CONCLUSIONS: Based on a review of a selected cohort of 105 TOF patients, the prevalence of mild CAD and significant CAD was 19% and 15%, respectively. Significant CAD was an independent risk factor for mortality. There is need for more research to determine optimal noninvasive diagnostic strategies and optimal patient selections and methods for revascularization.

Outcomes of Anticoagulation Therapy in Adults With Tetralogy of Fallot.

Background Available outcomes data for anticoagulation therapy in adults with congenital heart disease ( CHD ) provide assessment of global risk of this therapy for CHD patients (a heterogeneous population), but the risk of complications for the different CHD diagnoses is unknown. The purpose of the study was to describe the indications for anticoagulation, and the incidence and risk factors for major bleeding complication in adults with tetralogy of Fallot. Methods and Results We queried Mayo Adult Congenital Heart Disease (MACHD) database for tetralogy of Fallot patients (aged ≥18 years) that received anticoagulation, 1990-2017. Of 130 patients (42±14 years, 75 men [58%]), warfarin and direct oral anticoagulants were used in 125 (96%) and 5 (4%), respectively because atrial arrhythmias (n=109), mechanical prosthetic valve (n=29), intracardiac thrombus (n=4), pulmonary embolism (n=6), stroke (n=3), and perioperative anticoagulation (n=44). The median hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score for the entire cohort was 1 (0-2) and 27 (21%) had hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2. There were 14 minor bleeding events (1.6% per year) and 11 major bleeding events (1.3% per year) in 8 patients during median follow-up of 74 months (856 patient-years). Mechanical prosthesis (hazard ratio 1.78, CI 1.29-3.77, P=0.021) and hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2 (hazard ratio 1.41, CI 1.03-3.88, P=0.046) were risk factors for major bleeding events. All-cause mortality was higher in patients with major bleeding events (n=6, 75%) compared with patients without major bleeding events (n=25, 21%), P=0.001. Conclusions Considering the heterogeneity of the CHD population, data from the current study may be better suited for clinical decision-making in tetralogy of Fallot patients.