Long Term Veno-Pulmonary Arterial Extracorporeal Membrane Oxygenation as Bridge To Lung Transplantation And Its Challenges: A Case Report.

We present a case of a patient with acute respiratory distress syndrome due to severe acute respiratory syndrome coronavirus 2 infection who underwent an extracorporeal membrane oxygenation (ECMO) run of 8 months with various configuration changes, including off-label use of cannulas. The patient eventually underwent successful double lung transplantation after a follow-up of 17 months, demonstrating the successful application of a hybrid approach and careful monitoring in the face of supply shortages during the pandemic. This case highlights the challenges faced by ECMO and transplantation centers during the pandemic and the importance of careful communication and planning to optimize patient outcomes.

Screening and diagnostic imaging at centres performing congenital heart surgery in middle-income countries.

BACKGROUND: Surgical care for CHD is increasingly available in low- and middle-income countries, and efforts to optimise outcomes are growing. This study characterises cardiac imaging and prenatal diagnosis infrastructure in this setting. METHODS: An infrastructure survey was administered to sites participating in the International Quality Improvement Collaborative for CHD. Questions regarding transthoracic, transesophageal and epicardial echocardiography, cardiac CT, cardiac magnetic resonance, prenatal screening and fetal echocardiography were included. Associations with in-hospital and 30-day mortality were assessed. RESULTS: Thirty-seven sites in 17 countries responded. Programme size and geography varied considerably: < 250 cases (n = 13), 250-500 cases (n = 9), > 500 cases (n = 15); Americas (n = 13), Asia (n = 18), and Eastern Europe (n = 6). All had access to transthoracic echo. Most reported transesophageal and epicardial echocardiography availability (86 and 89%, respectively). Most (81%) had cardiac CT, but only 54% had cardiac magnetic resonance. A third reported impediments to imaging, including lack of portable machines, age/size-appropriate equipment and advanced cardiac imaging access and training. Only 19% of centres reported universal prenatal CHD screening in their catchment area, and only 46% always performed fetal echocardiography if screening raised concern for CHD. No statistically significant associations were identified between imaging modality availability and surgical outcomes. CONCLUSIONS: Although access to echocardiography is available in most middle-income countries; advanced imaging modalities (cardiac CT and magnetic resonance) are not always accessible. Prenatal screening for CHD is low, and availability of fetal echocardiography is limited. Imaging infrastructure in low- and middle-income countries and associations with outcomes merits additional study.

An Unusual Cause of Right Heart Dysfunction and High Output Heart Failure in a Young Woman.

A 35-year-old female presented to our emergency department with clinical signs of acute heart failure. Clinical workup identified severe right heart (RH) dilation and dysfunction with a crossing membrane structure in the right atrium. Right heart catheterization confirmed high output heart failure (HOHF), pulmonary hypertension (PH), and left-to-right blood shunting followed by the documentation of multiple liver and pulmonary arteriovenous malformations (AVMs). Hereditary Hemorrhagic Telangiectasia (HHT) diagnosis was made according to clinical criteria and was established as the cause of HOHF because of chronic volume overload from systemic to pulmonary shunts. With this illustrative case, we aim to discuss the broad spectrum of clinical manifestations of HHT and the unusual phenotype of HOHF secondary to HHT. This case also highlights the broad diagnosis of atrial echocardiographic abnormalities and cardiac structural distortion secondary to high output that can be misleading at imaging evaluation.

Nephrogenic Systemic Fibrosis in Patients with Chronic Kidney Disease after the Use of Gadolinium-Based Contrast Agents: A Review for the Cardiovascular Imager.

Gadolinium-enhanced cardiac magnetic resonance has revolutionized cardiac imaging in the last two decades and has emerged as an essential and powerful tool for the characterization and treatment guidance of a wide range of cardiovascular diseases. However, due to the high prevalence of chronic renal dysfunction in patients with cardiovascular conditions, the risk of nephrogenic systemic fibrosis (NSF) after gadolinium exposure has been a permanent concern. Even though the newer macrocyclic agents have proven to be much safer in patients with chronic kidney disease and end-stage renal failure, clinicians must fully understand the clinical characteristics and risk factors of this devastating pathology and maintain a high degree of suspicion to prevent and recognize it. This review aimed to summarize the existing evidence regarding the physiopathology, clinical manifestations, diagnosis, and prevention of NSF related to the use of gadolinium-based contrast agents.

45,X/46,XY Mosaicism with Male Phenotype: Case Report.

Mixed gonadal dysgenesis is the most common chromosomal abnormality with ambiguous genitalia, defined as a 45,X/46,XY mosaicism. It can present with a normal male phenotype, ambiguous genitalia, or features of Turner syndrome. A 14-year-old patient was referred to the genetics clinic due to hypospadia, cryptorchidism, and aortic coarctation. During the physical examination, short stature, webbed neck, and Blashko lines on his back were noted. He had a previous karyotype reported as normal. However, due to an inadequate evolution and a low resolution on the previous test, a higher resolution karyotype was performed, identifying a mosaicism 45,X/46,XY. A multidisciplinary board examined the case, and follow-up with tumor markers was carried out to evaluate the presence of gonadoblastoma, one of the main complications in these patients. Treatment should be transdisciplinary and focused on the particular characteristics of each case. Other treatment alternatives include corrective surgery and hormonal therapy.

Chagas Cardiomyopathy: From Romaña Sign to Heart Failure and Sudden Cardiac Death.

Despite nearly a century of research and accounting for the highest disease burden of any parasitic disease in the Western Hemisphere, Chagas disease (CD) is still a challenging diagnosis, primarily due to its poor recognition outside of Latin America. Although initially considered endemic to Central and South America, globalization, urbanization, and increased migration have spread the disease worldwide in the last few years, making it a significant public health threat. The international medical community's apparent lack of interest in this disease that was previously thought to be geographically restricted has delayed research on the complex host-parasite relationship that determines myocardial involvement and its differential behavior from other forms of cardiomyopathy, particularly regarding treatment strategies. Multiple cellular and molecular mechanisms that contribute to degenerative, inflammatory, and fibrotic myocardial responses have been identified and warrant further research to expand the therapeutic arsenal and impact the high burden attributed to CD. Altogether, cardiac dysautonomia, microvascular disturbances, parasite-mediated myocardial damage, and chronic immune-mediated injury are responsible for the disease's clinical manifestations, ranging from asymptomatic disease to severe cardiac and gastrointestinal involvement. It is crucial for healthcare workers to better understand CD transmission and disease dynamics, including its behavior on both its acute and chronic phases, to make adequate and evidence-based decisions regarding the disease. This review aims to summarize the most recent information on the epidemiology, pathogenesis, clinical presentation, diagnosis, screening, and treatment of CD, emphasizing on Chagasic cardiomyopathy's (Ch-CMP) clinical presentation and pathobiological mechanisms leading to sudden cardiac death.

Síndrome DRESS asociado a fenitoína: informe de caso / DRESS syndrome associated with phenytoin: case report

El síndrome DRESS es una reacción adversa dermatológica que puede presentarse debido a diversos medicamentos, y constituye uno de los diagnósticos más importantes por encima del síndrome de Stevens-Johnson. Se trata de un caso relacionado con una reacción adversa de muy baja frecuencia, que está documentada en la literatura científica, a varios medicamentos, entre ellos la fenitoína. Por lo mencionado, la publicación de estos casos resulta escasa y limitada. Las principales preocupaciones del paciente relacionadas con su cuadro clínico radicaban en el gran compromiso cutáneo que lo llevó a hospitalización, dolor e incomodidad, por el cual recurrió al manejo tópico generalizado con vaselina. Los hallazgos clínicos relevantes fueron: eosinofilia severa, ulceraciones cutáneas, hepatitis química y fiebre. Con los hallazgos del cuadro clínico y la evaluación de la escala RegiSCAR se hace el diagnóstico de síndrome DRESS inducido por fenitoína. Se suspende la fenitoína, se inicia levetiracetam y se administran corticosteroides y acetaminofén con evolución favorable. (AU)

DRESS syndrome is a dermatological adverse reaction can occur due to various medications, being one of the most important diagnoses above Steven-Johnson syndrome. This is a case related to a very low frequency adverse reaction that is documented in the scientific literature to several medicines among those, the phenytoin. Therefore, the publication of these cases is scarce and limited. The main concerns of the patients related to their clinical picture were due to the great cutaneous compromise that lead to hospitalization, pain and discomfort for which they resorted to generalized topical management with vaseline (petrolatum). Relevant clinical findings were severe eosinophilia, skin ulcerations, chemical hepatitis and fever. With clinical picture findings and evaluation of the RegiSCAR scale, the diagnosis of Phenytoin-induced DRESS syndrome is made. Phenytoin is discontinued, levetiracetam is started and corticosteroids and acetaminophen are administrated with favorable evolution. (AU)

Hipercolesterolemia familiar: serie de 36 casos con fenotipo de hipercolesterolemia familiar homocigótica / Familial hypercholesterolaemia: A study of 36 cases with a phenotype of homozygous familiar hypercholesterolaemia

Resumen Introducción: La hipercolesterolemia familiar homocigótica (HFHo) se caracteriza por niveles muy elevados de cLDL y por enfermedad aterosclerótica temprana. Aunque la frecuencia es baja (1/300.000), las complicaciones son muy severas y pueden ser evitadas. Encontrar y tratar esta población de manera temprana podría reducir la mortalidad. Se describen 36 casos en Colombia, en donde se calcula que haya entre 160 y 200 casos. Resultados: Un total de 36 pacientes con fenotipo sugestivo de HFHo fueron identificados y tratados en un período de observación de cuatro años. La media de edad fue 27 años (24 mujeres). 34 pacientes tuvieron un puntaje según la Red de Clínicas de Lípidos de Holanda (RCLH) mayor de 8 (diagnóstico definitivo) y los restantes 2 tenían puntaje equivalente a diagnóstico probable. Un cuarto de los casos procedían de la costa norte colombiana. En las pruebas genéticas, 14 fueron homocigóticos verdaderos para mutación del gen que codifica para el receptor de LDL (LDLR), 12 heterocigóticos compuestos, 2 heterocigóticos dobles y uno autosómico recesivo (LDLRAP1); 5 pacientes fueron heterocigóticos simples (LDLR) y 2 pacientes no autorizaron la prueba. En los homocigóticos verdaderos, la variante más frecuente encontrada fue la c.11G>A. 14 pacientes cursaron con enfermedad coronaria, 9 con estenosis carotídea, 8 con estenosis aórtica y 2 tuvieron ataques cerebrovasculares (ACV). 34 pacientes recibían estatinas (24 rosuvastatina), 30 recibían ezetimibe, 2 recibían evolocumab y 20 recibían lomitapide (dosis promedio 12,7mg). Ninguno recibió aféresis de cLDL. Los medicamentos, en general, fueron bien tolerados y la reducción promedio de cLDL con la terapia fue de 533,7mg/dl a 245,1mg/dl (54%). Conclusiones: Todos los pacientes recibieron tratamiento hipolipemiante y se encontraron alteraciones genéticas diagnósticas en todos aquellos que autorizaron el examen. Los niveles elevados de cLDL conllevan tanto riesgo que el tratamiento debe establecerse aún sin conocer el diagnóstico genético.

Abstract Background: Homozygous familial hypercholesterolemia (HoFH) is characterized for very high levels of cLDL and early cardiovascular disease. Although incidence is low (1/300 000), complications are very severe and can be avoided. Finding and treating this population promptly could reduce mortality. We describe 36 cases in Colombia, where 160 to 200 cases are expected. Results: 36 patients with phenotype of HoHF were identified and treated in a follow-up of 4 years. The mean age was 27 years (24 women). 34 of them had at least 8 points in the FH Dutch Lipid Clinic Criteria (definitive diagnosis) and two had probable diagnosis. A quarter of the cases came from the Colombian North Coast. In molecular tests, 14 were true homozygous for LDLR, 12 were compound heterozygous for LDLR, 2 double heterozygous and one was autosomal recessive; 5 were heterozygous and 2 patients did not authorized genetic test. In true homozygous subjects, the most frequent variant was c.11G>A. 14 patients had coronary disease, 9 carotid stenosis, 8 aortic stenosis and 2 had stroke. 34 patients were on statins (25 rosuvastatin), 30 were receiving ezetimibe, 2 were receiving a PSCK9 inhibitor (evolocumab) and 20 were on lomitapide with mean doses of 12.7mg. None received lipoprotein apheresis. Medications were very well tolerated. Changes in cLDL after therapy was from 533.7 mg/dL to 245 mg/dL, (54%). Conclusions: Treatment was started in all patients. We found genetic mutations in all patients with genetic tests. The high levels of cLDL mean such a high risk that treatment must be started promptly, even without a genetic test.

Cardiovascular Magnetic Resonance Imaging Evidence of Edema in Chronic Chagasic Cardiomyopathy.

The persistence of inflammatory processes in the myocardium in varying degrees of chronic Chagas heart disease has been poorly investigated. We hypothesized that edema could occur in patients with chronic chagasic cardiomyopathy and corresponds to the persistence of inflammatory processes in the myocardium. Eighty-two Chagas disease (CD) seropositive patients (64.6% females; age = 58.9 ± 9.9) without ischemic heart disease or conditions that cause myocardial fibrosis and dilation were considered. Late gadolinium enhancement (LGE) and T2-weighted magnetic resonance imaging of edema were obtained and represented using a 17-segment model. Patients were divided into three clinical groups according to the left ventricular (LV) ejection fraction (EF) as G1 (EF > 60%; n=37), G2 (35% > EF < 60%; n=33), and G3 (EF < 35%; n=12). Comparisons were performed by the Fisher or ANOVA tests. Bonferroni post hoc, Spearman correlation, and multiple correspondence analyses were also performed. Edema was observed in 8 (9.8%) patients; 2 (5.4%) of G1, 4 (12.1%) of G2, and 2 (16.7%) of G3. It was observed at the basal inferolateral segment in 7 (87.5%) cases. LGE was observed in 48 (58.5%) patients; 16 (43.2%) of G1, 21 (63.6%) of G2, and 11 (91.7%) of G3 (p < 0.05). It was observed in the basal inferior/inferolateral/anterolateral segments in 35 (72.9%) patients and in the apical anterior/inferior/lateral and apex segments in 21 (43.7%), with midwall (85.4%; n=41), subendocardial (56.3%; n=27), subepicardial (54.2%; n=26), transmural (31.2%; n=15), and RV (1.2%; n=1) distribution. Subendocardial lesions were observed only in patients with LVEF < 35%. There was no involvement of the mid-inferolateral/anterolateral segments with an LVEF > 35% (p < 0.05). Deteriorations of the LV and RV systolic functions were positively correlated (r s =0.69; p < 0.05) without evidence of LGE in the RV. Edema can be found in patients with chagasic cardiomyopathy in the chronic stage. In later stages of cardiac dilation with low LVEF, the LGE pattern involves subendocardium and mid locations. Deteriorations of RV and LV are positively correlated without evidence of fibrosis in the RV.

Patterns of CMR measured longitudinal strain and its association with late gadolinium enhancement in patients with cardiac amyloidosis and its mimics.

BACKGROUND: Regional variability of longitudinal strain (LS) has been previously described with echocardiography in patients with cardiac amyloidosis (CA), however, the reason for this variability is not completely evident. We sought to describe regional patterns in LS using feature-tracking software applied to cardiovascular magnetic resonance (CMR) cine images in patients with CA, hypertrophic cardiomyopathy (HCM), and Anderson-Fabry's disease (AFD) and to relate these patterns to the distribution of late gadolinium enhancement (LGE). METHODS: Patients with CA (n = 45) were compared to LV mass indexed matched patients with HCM (n = 19) and AFD (n = 19). Peak systolic LS measurements were obtained using Velocity Vector Imaging (VVI) software on CMR cine images. A relative regional LS ratio (RRSR) was calculated as the ratio of the average of the apical segmental LS divided by the sum of the average basal and mid-ventricular segmental LS. LGE was quantified for the basal, mid, and apical segments using a threshold of 5SD above remote myocardium. A regional LGE ratio was calculated similar to RRSR. RESULTS: Patients with CA had significantly had worse global LS (-15.7 ± 4.6%) than those with HCM (-18.0 ± 4.6%, p = 0.046) and AFD (-21.9 ± 5.1%, p < 0.001). The RRSR was higher in patients with CA (1.00 ± 0.31) than in AFD (0.79 ± 0.24; p = 0.018) but not HCM (0.84 ± 0.32; p = 0.114). In CA, a regional difference in LGE burden was noted, with lower LGE in the apex (31.5 ± 19.1%) compared to the mid (38.2 ± 19.0%) and basal (53.7 ± 22.7%; p < 0.001 for both) segments. The regional LGE ratio was not significantly different between patients with CA (0.33 ± 0.15) and AFD (0.47 ± 0.58; p = 0.14) but lower compared to those with HCM (0.72 ± 0.43; p < 0.0001). LGE percentage showed a significant impact on LS (p < 0.0001), with a 0.9% decrease in absolute LS for every 10% increase in LGE percentage. CONCLUSION: The presence of marked "relative apical sparing" of LS along with a significant reduction in global LS seen in patients with CA on CMR cine analysis may provide an additional tool to differentiate CA from other cause of LVH. The concomitant presence of a base to apex gradient in quantitative LGE burden suggests that the regional strain gradient may be at least partially explained by the burden of amyloid deposition and fibrosis.

Hallazgos imagenológicos en cálculos abandonados en cavidad abdominal después de colecistectomía laparoscópica: serie de 7 casos y revisión de la literatura / Imaging Findings of Stones Left in the Abdominal Cavity Following Laparascopic Cholecystectomy: A Series of Seven Cases and Literature Review

Resumen La colecistectomía laparoscópica (CL) es la técnica quirúrgica de elección actual para el tratamiento de la colelitiasis sintomática. Sin embargo, este procedimiento acarrea ciertas complicaciones, dentro de las cuales destacamos la perforación de la vesícula biliar con subsiguiente caída de los cálculos y bilis a la cavidad peritoneal, que puede dar origen a la formación de abscesos abdominales tiempo después de dicho procedimiento quirúrgico. Por tal motivo, los cálculos biliares abandonados deben reconocerse como una fuente potencial para la formación de un proceso inflamatorio intraabdominal, en especial cuando se tiene historia de perforación vesicular durante el procedimiento, o en los casos de conversión a colecistectomía abierta y caída de los cálculos a la cavidad peritoneal. Tanto los radiólogos como los cirujanos deben familiarizarse con los hallazgos imagenológicos de las diferentes modalidades diagnósticas, como el ultrasonido, la TC y la resonancia magnética, así como con las diferentes manifestaciones clínicas que pueden presentarse. En esta publicación, presentamos los casos de 7 pacientes con antecedente de colecistectomía laparoscópica que acudieron al servicio de urgencia de nuestro hospital por dolor abdominal y en quienes los hallazgos imagenológicos determinaron la presencia de colecciones perihepáticas por cálculos caídos.

Abstract Laparascopic cholecystectomy (LC) is the current surgical technique of choice for the treatment of symptomatic cholelithiasis. However, this procedure leads to certain complications, of which we highlight gallbladder perforation with the subsequent spillage of stones and bile into the peritoneal cavity, which may give rise to the formation of abdominal abscesses some time after the surgery. For this reason, abandoned gallstones should be recognized as a potential source of intra-abdominal inflammatory processes, especially when there is a history of gallbladder perforation during the procedure, or in cases of conversion to open cholecystectomy and spillage of gallstones into the peritoneal cavity. Both radiologists as well as surgeons should be familiar with the imaging findings of the various diagnostic modalities such as ultrasound, CT and magnetic resonance imaging, as well as the various clinical manifestations that can present. In this publication we present 7 cases of patients with a history of laparascopic cholecystectomy who presented to our hospital´s ER with abdominal pain and in whom the imaging findings showed the presence of perihepatic collections caused by spilled stones.

Randomized controlled trial of relaxation music to reduce heart rate in patients undergoing cardiac CT.

OBJECTIVES: To evaluate the heart rate lowering effect of relaxation music in patients undergoing coronary CT angiography (CCTA), pulmonary vein CT (PVCT) and coronary calcium score CT (CCS). METHODS: Patients were randomised to a control group (i.e. standard of care protocol) or to a relaxation music group (ie. standard of care protocol with music). The groups were compared for heart rate, radiation dose, image quality and dose of IV metoprolol. Both groups completed State-Trait Anxiety Inventory anxiety questionnaires to assess patient experience. RESULTS: One hundred and ninety-seven patients were recruited (61.9 % males); mean age 56y (19-86 y); 127 CCTA, 17 PVCT, 53 CCS. No significant difference in heart rate, radiation dose, image quality, metoprolol dose and anxiety scores. 86 % of patients enjoyed the music. 90 % of patients in the music group expressed a strong preference to have music for future examinations. The patient cohort demonstrated low anxiety levels prior to CT. CONCLUSION: Relaxation music in CCTA, PVCT and CCS does not reduce heart rate or IV metoprolol use. Patients showed low levels of anxiety indicating that anxiolytics may not have a significant role in lowering heart rate. Music can be used in cardiac CT to improve patient experience. KEY POINTS: • Relaxation music does not reduce heart rate in cardiac CT • Relaxation music does not reduce beta-blocker use in cardiac CT • Relaxation music has no effect on cardiac CT image quality • Low levels of anxiety are present in patients prior to cardiac CT • Patients enjoyed the relaxation music and this results in improved patient experience.

Manejo endovascular de pseudoaneurismas: presentación de dos casos / Endovascular management of pseudoaneurysms: presentation of two cases

Los pseudoaneurismas son dilataciones debidas a la disrupción de una o más capas de la pared arterial, con fuga sanguínea a través del defecto que está contenida por el tejido más externo e intacto de las capas media y adventicia. El reparo de los pseudoaneurismas debe realizarse para reducir el riesgo complicaciones. Tradicionalmente el manejo de elección en este tipo de lesiones se realizaba quirúrgicamente, con mayor probabilidad de sangrado o lesión neurológica. En la actualidad el manejo endovascular es de elección en pacientes seleccionados. En este artículo se reportan dos casos, para demostrar las diferentes técnicas de tratamiento endovascular en pseudoaneurismas de origen traumático.

Pseudoaneurysms are dilatations due to a disruption of one or more layers of an arterial wall with blood leakage through the defect that is contained by the outer tissue layers of an intact media and adventitia. A pseudoaneurysm must be repaired to avoid the risk of complications. Traditionally they were repaired surgically with a greater risk of bleeding and nerve injury At present, endovascular management is preferred in selected patients. This article reports two cases to illustrate the different techniques of endovascular treatment oftraumatic pseudoaneurysms.