HETEROTAXY SYNDROME IN COVID-19 PATIENT WITH PULMONARY EMBOLISM AND AORTIC ANEURYSM: A RARE ACCIDENTAL COMBINATION (RADIOLOGICAL ASSESSMENT). / GETEROTAKSIIa, TROMBOEMBOLIIa LEGENEVOÏ ARTERIÏ TA ANEVRYZMA AORTY: RIDKISNE VYPADKOVE POIeDNANNIa (RADIOLOGIChNA OTsINKA).

With limited time for analysing computed tomography (CT) data in medical emergencies, radiologists and clinicians are not always aware of congenital pathologies, especially in asymptomatic cases. We present a case report of heterotaxy syndrome detected incidentally in a 62-year-old female with massive pulmonary embolism, local saccular aneurysm of the aortic arch and COVID-19. The presented case describes an unusual combination of life-threatening conditions and congenital anomalies. Using the CT angiography, we reported the preserved topography and morphology of the heart chambers. The superior and inferior venae cavae were located on the left, draining into a significantly dilated coronary sinus. Disordered abdominal organ arrangement included midline liver, right-sided stomach, the central location of the short pancreas, malrotation of the intestine, polysplenia, and atypical rise of visceral branches of the abdominal aorta. A comprehensive assessment of CT images promoted verification of undiagnosed congenital anomalies. Prompt recognition of heterotaxy syndrome in life-threatening conditions significantly impacts treatment tactics and should be the course of diagnostic and cautious interventions to reduce further complications.

Discovery of novel biomarkers for atherosclerotic aortic aneurysm through proteomics-based assessment of disease progression.

Since aortic aneurysms (AAs) are mostly asymptomatic, but they have a high mortality rate upon rupture, their detection and progression evaluation are clinically important issues. To discover diagnostic biomarkers for AA, we performed proteome analysis of aortic media from patients with thoracic atherosclerotic AA (TAAA), comparing protein levels between the aneurysm and normal tissue areas. After hierarchical clustering analysis of the proteome analysis data, tissue samples were classified into three groups, regardless of morphological features. This classification was shown to reflect disease progression stage identified by pathological examination. This proteomics-based staging system enabled us to identify more significantly altered proteins than the morphological classification system. In subsequent data analysis, Niemann-Pick disease type C2 protein (NPC2) and insulin-like growth factor-binding protein 7 (IGFBP7) were selected as novel biomarker candidates for AA and were compared with the previously reported biomarker, thrombospondin 1 (THBS1). Blood concentrations of NPC2 and IGFBP7 were significantly increased, while THBS1 levels were decreased in TAAA and abdominal atherosclerotic AA patients. Receiver operating characteristic analysis of AA patients and healthy controls showed that NPC2 and IGFBP7 have higher specificity and sensitivity than THBS1. Thus, NPC2 and IGFBP7 are promising biomarkers for the detection and progression evaluation of AA.

Duplex Ultrasound Assessment and Outcomes of Renal Malperfusion Syndromes after Acute Aortic Dissection.

BACKGROUND: We investigated the feasibility of renal duplex ultrasound in the identification of renal malperfusion in acute aortic dissection and evaluated whether intervention for renal malperfusion improved outcomes over best medical management alone. METHODS: All patients with acute aortic dissections involving the renovisceral aorta who underwent a duplex ultrasound were included (2004-2016). We assessed duplex findings among patients who developed acute kidney injury (AKI; 50% increase in serum creatinine) and compared AKI, 30-day mortality, and overall survival among patients who underwent a procedure to treat malperfusion versus those who did not. RESULTS: Of 37 patients with acute dissection involving the renovisceral aorta (73% were male, 59% had type B dissection, mean follow-up 4.6 ± 0.6 years), 70% developed AKI, 11% required dialysis, and 5% developed permanent dialysis dependence. AKI was correlated with higher peak creatinine levels (4.2 vs. 2.2 mg/dL, P < 0.001), although 30-day mortality and overall survival were similar (both, P ≥ 0.24). Progression to AKI was associated with significantly lower end-diastolic velocity (EDV) measurements on renal duplex (17 vs. 27 cm/sec, P = 0.03); an EDV threshold of 23 cm/sec had a positive predictive value of 85% for AKI. Operative intervention (n = 10) was associated with lower follow-up creatinine (0.9 vs. 2.1 mg/dL, P = 0.002), although there was no difference in progression to dialysis dependence, 30-day mortality, or overall survival (all, P ≥ 0.34). CONCLUSIONS: Patients who developed AKI demonstrated characteristic renal duplex ultrasound findings with lower EDV measurements in the distal renal arteries bilaterally. Performing a renal malperfusion procedure was associated with normalization of postoperative creatinine without affecting 30-day mortality or overall survival.

Ethnic disparities in outcomes of patients with complicated type B aortic dissection.

OBJECTIVE: The objective of this study was to evaluate the difference in outcomes after endovascular intervention in patients with complicated type B aortic dissection (TBAD) based on ethnicity and blood pressure control. METHODS: Between 2012 and 2016, there were 126 patients who underwent endovascular procedures for complicated TBAD at a single-institution quaternary referral center. Patients self-identified as African American (n = 53), white (n = 70), and Asian (n = 3). African American and white patients were compared on a number of variables, including age, ethnicity, insurance type, blood pressure, comorbidities, number of previous interventions, and number of antihypertension medications they were taking before intervention. Primary outcomes were survival and need for reintervention. RESULTS: Kaplan-Meier estimates for survival for African Americans vs whites were 94% vs 89%, 91% vs 83%, 89% vs 79%, and 89% vs 76% at 30 days, 1 year, 3 years, and 5 years, respectively (P = .05). African Americans were younger overall (52.5 ± 11 years) vs whites (63.7 ± 14.7 years; P < .0001). African Americans required a significantly greater number of reinterventions (P = .007). They also had higher rates of chronic kidney disease (P = .01), smoking (P = .03), and cocaine use (P = .02) and were more likely to be on Medicaid (P = .02). Hypertension was poorly controlled in both groups, with the percentage of patients with uncontrolled hypertension (systolic >140 mm Hg) preoperatively, postoperatively, and 30 days after intervention at 32%, 32%, and 39%. There was no significant difference between the cohorts in uncontrolled hypertension preoperatively (P = .39) or postoperatively (P = .63). However, more African Americans had uncontrolled hypertension at 30 days (African Americans, 49%; whites, 31%; odds ratio, 2.1; P = .09). African Americans were taking a greater number of antihypertension medications at presentation than whites (P = .01) and specifically had higher use rates of beta blockers (P = .02), diuretics (P = .02), and angiotensin-converting enzyme inhibitors (P = .04). CONCLUSIONS: African Americans with TBAD present at a younger age than their white counterparts do and have a survival advantage up to at least 5 years. However, African Americans have a higher rate of reintervention that is probably associated with poor blood pressure control despite taking more antihypertension medications both before and after the repair. It appears that optimal medical therapy is difficult to achieve in all groups. More aggressive medical management is needed, particularly more so in African Americans, which may in turn decrease the number of interventions and potentially improve long-term survival.

Routine transfusion of platelet concentrates effectively reduces reoperation rate for bleeding and pericardial effusion after elective operations for ascending aortic aneurysm.

Patients with ascending aortic aneurysm undergoing complex surgical procedures are at increased risk of early postoperative excessive blood loss. The aim of this study was to analyze safety and efficacy of routine transfusions of platelet (PLT) concentrates in reduction of hemorrhagic postoperative complications. The study involved 396 consecutive patients (289 males and 107 females) with the mean age of 55.9 ± 13.6 years who underwent elective operations for aortic aneurysms. They were divided retrospectively into two groups, without (group A; n = 123) or with the routine use of PLTs (group B; n = 273). PLTs were transfused intraoperatively just after completion of cardiopulmonary bypass. Twelve patients in group A (9.8%) and 10 (3.7%) in group B required re-thoracotomy due to hemorrhage (p = 0.027). Routine transfusions of PLT concentrates reduced postoperative incidence of excessive pericardial effusion from 24.1% in group A to 2.1% in group B (p = 0.002). In a consequence, significantly less units (p < 0.0001) of red blood concentrates and fresh frozen plasma were transfused in group B than in group A. The rates of other adverse events in the early postoperative period did not differ between groups. Patients with pericardial effusion required 6.3 ± 2.7 additional days of hospitalization due to surgical re-intervention. Neither blood transfusion-related infections nor adverse reactions were noted. In conclusion, routine intraoperative transfusions of PLT concentrates in patients with ascending aortic aneurysms significantly reduced a need for re-intervention due to both early bleeding and late cardiac tamponade.

Rationale and design of GISSI OUTLIERS VAR Study in bicuspid aortic valve patients: prospective longitudinal, multicenter study to investigate correlation between surgical, echo distinctive features, histologic and genetic findings in phenotypically homogeneous outlier cases.

BACKGROUND/OBJECTIVES: Bicuspid aortic valve (BAV) is the most common congenital heart disorder, affecting up to 2% of the population. Involvement of aortic root and ascending aorta (aneurysm or, eventually, dissection) is frequent in patients with pathologic or normal functioning BAV. Unfortunately, there are no well-known correlations between valvular and vascular diseases. In VAR protocol, with a new strategy of research, we analysemultiple aspects of BAV disease through correlation between surgical, echo, histologic and genetic findings in phenotypically homogeneous outlier cases. METHODS: VAR protocol is a prospective, longitudinal, multicenter study. It observes 4 homogeneous small groups of BAV surgical patients (15 patients each): isolated aortic regurgitation, isolated ascending aortic aneurysm, aortic regurgitation associated with aortic aneurysm, isolated aortic stenosis in older patients (>60years). Echo analysis is extended to first-degree relatives and, in case of BAV, genetic test is performed. Patients and relatives are enrolled in 10 cardiac surgery/cardiologic centers throughout Italy. CONCLUSIONS: The aim of the study is to identify predictors of favorable or unfavorable evolution of BAV in terms of valvular dysfunction and/or aortic aneurysm. Correlations between different features could help in identification of various BAV risk groups, rationalizing follow-up and treatment.

Cost-effectiveness of endovascular versus open repair of acute complicated type B aortic dissections.

OBJECTIVE: This study weighed the cost and benefit of thoracic endovascular aortic repair (TEVAR) vs open repair (OR) in the treatment of an acute complicated type B aortic dissection by (TBAD) estimating the cost-effectiveness to determine an optimal treatment strategy based on the best currently available evidence. METHODS: A cost-utility analysis from the perspective of the health system payer was performed using a decision analytic model. Within this model, the 1-year survival, quality-adjusted life-years (QALYs), and costs for a hypothetical cohort of patients with an acute complicated TBAD managed with TEVAR or OR were evaluated. Clinical effectiveness data, cost data, and transitional probabilities of different health states were derived from previously published high-quality studies or meta-analyses. Probabilistic sensitivity analyses were performed on uncertain model parameters. RESULTS: The base-case analysis showed, in terms of QALYs, that OR appeared to be more expensive (incremental cost of €17,252.60) and less effective (-0.19 QALYs) compared with TEVAR; hence, in terms of the incremental cost-effectiveness ratio, OR was dominated by TEVAR. As a result, the incremental cost-effectiveness ratio (ie, the cost per life-year saved) was not calculated. The average cost-effectiveness ratio of TEVAR and OR per QALY gained was €56,316.79 and €108,421.91, respectively. In probabilistic sensitivity analyses, TEVAR was economically dominant in 100% of cases. The probability that TEVAR was economically attractive at a willingness-to-pay threshold of €50,000/QALY gained was 100%. CONCLUSIONS: The present results suggest that TEVAR yielded more QALYs and was associated with lower 1-year costs compared with OR in patients with an acute complicated TBAD. As a result, from the cost-effectiveness point of view, TEVAR is the dominant therapy over OR for this disease under the predefined conditions.

Comparison of immediate results and mid-term follow-up of surgical and percutaneous closure of ruptured sinus of Valsalva aneurysm.

BACKGROUND: The past decade has witnessed considerable improvement in implantation techniques for percutaneous closure of ruptured sinus of Valsalva aneurysm. This study was undertaken to compare the immediate results and mid-term follow-up of traditional surgery and percutaneous closure of ruptured sinus of Valsalva aneurysm in China. METHODS: Between January 1993 and January 2013, a total of 35 patients with ruptured sinus of Valsalva aneurysm were enrolled for attempted closure in our institution. Twenty patients (57.1%) underwent surgical repair and the other 15 patients (42.9%) underwent percutaneous closure. RESULTS: Nineteen patients (95.0%) in the surgical group had complete closure and 14 patients (93.3%) in the percutaneous closure group were treated successfully (p=0.681). Twelve patients with ruptured sinus of Valsalva aneurysm combined with subarterial ventricular septal defect (VSD) underwent surgical repair concomitantly. Two patients in the percutaneous closure group were treated for ruptured sinus of Valsalva aneurysm and perimembranous VSD using occluders concomitantly. One case of severe hemolysis developed 8h after the procedure in the percutaneous closure group. One death and one case of patch leak occurred in the surgical group during hospitalization. The time of hospital stay in the percutaneous closure group was shorter than that in the surgical group (p=0.004). The total medical cost of isolated ruptured sinus of Valsalva aneurysm in the percutaneous closure group was lower compared with that in the surgical group (p=0.046). During a median follow-up of 15 months (2-240 months), there were no deaths, infective endocarditis, device embolization, or malposition. CONCLUSIONS: Percutaneous closure is an attractive alternative to surgery in selected patients with ruptured sinus of Valsalva aneurysm for the better clinical advantages and economic benefits.

Two- and three-dimensional transthoracic echocardiography in the assessment of acquired ascending aortic aneurysm to pulmonary artery fistula.

Aorta to pulmonary artery fistula is an uncommon and potentially fatal condition. This case is of a 48-year-old Caucasian male with congestive heart failure and multiple aortic valve replacement surgeries who presented with an acquired ascending aortic aneurysm to pulmonary artery fistula diagnosed using two-dimensional transthoracic echocardiography via nonstandard imaging windows. Three-dimensional transthoracic echocardiography using live/real time three-dimensional color Doppler was used to assess the size of the opening of the fistula, providing additional value. This patient was surgically managed and is doing well 8 months postoperation.

Observational study of mortality risk stratification by ischemic presentation in patients with acute type A aortic dissection: the Penn classification.

BACKGROUND: Acute type A aortic dissection is a surgical emergency, with an operative mortality as high as 25%. Ischemia is a known predictor of mortality. We tested the efficacy of a classification system--the Penn classification, which is based on ischemic pattern at clinical presentation--to stratify operative mortality risk and identify high-risk groups of patients for further intervention and study. METHODS: In this prospective observational study, patients underwent a standard aortic dissection repair protocol at the University of Pennsylvania, Philadelphia, PA, from 1993 to 2004. Patients were classified as having no ischemia, branch vessel malperfusion with localized organ ischemia, generalized ischemia with circulatory collapse, with or without cardiac involvement, or a combination of localized and generalized ischemia. RESULTS: The cohort comprised 221 patients. The mean age was 61.6 (+/- 14.8) years and 66.5% were male. At presentation 57.9% of patients had no ischemia, 17.6% had localized ischemia, 15.4% had generalized ischemia, and 9.0% had both localized and generalized ischemia. Overall, 28 (12.7%) patients died during the perioperative period. All-cause mortality differed significantly between groups (no ischemia 3.1%, localized ischemia 25.6%, generalized ischemia 17.6%, combined ischemia 40.0%), yielding an overall 8.3-fold difference for no compared with any ischemia (3.1% versus 25.8%, P = 0.0001). Ischemic presentations together accounted for 85.7% of all deaths. CONCLUSION: The Penn classification of acute type A aortic dissection enabled stratification of patients by operative mortality risk. The system requires further validation, but might facilitate new ways to analyze mortality data for this disorder.

Transluminal hypogastric artery occlusion with an Amplatzer vascular plug during endovascular aortic aneurysm repair.

OBJECTIVE: To evaluate our initial experience with hypogastric artery occlusion using a nitinol vascular plug during endovascular aortic aneurysm repair (EVAR). METHODS: We reviewed the records and images of 23 consecutive patients who underwent transluminal vessel occlusion of the hypogastric artery with a nitinol plug, as well as a cohort of 19 patients who underwent hypogastric artery embolization with coils in conjunction with EVAR. RESULTS: There were no demographic differences between the two groups of patients. Hypogastric artery occlusion was successful in all cases when a nitinol vascular plug was used. When coils were used, there was one unsuccessful embolization which required a second procedure. The number of embolic devices used in the coil group was 7.53 (range, three to 13) compared with 1.35 (range, one to six) in the plug group (P < .05). Only one plug was used in 19 of 23 cases. The average cost to embolize per hypogastric artery was $1,496 compared with $470 when a nitinol plug was used. There were two instances of coil migration. No other intraoperative complications occurred. At one month follow up, seven patients (35%) in the coil group complained of buttock claudication compared with two patients (9%) in the nitinol plug group (P = .027). CONCLUSION: Our experience demonstrates the safety and effectiveness of the nitinol vascular plug for hypogastric artery occlusion during EVAR. When compared with coils for hypogastric embolization during EVAR, nitinol vascular plugs are less expensive, produce less technical complications, and are associated with a significantly lower incidence of gluteal claudication.

Assessment of aortic dissection and hematoma.

Aortic dissection and aortic intramural hematoma are highly lethal diseases occurring mostly in patients with arterial hypertension or Marfan syndrome. Transesophageal echocardiography (TEE) is the diagnostic imaging procedure of choice in the unstable patient. Hallmarks of dissection are the presence of an intimal membrane, or flap, dividing a true and a false lumen, entry and reentry tears, periaortic hematoma, pericardial effusion or tamponade, and aortic regurgitation in dissection of the ascending aorta. TEE allows detection of involvement of the ascending aorta, which places the patient in an extremely high-risk group and necessitates urgent operation. The accuracy of TEE is similar to computed tomography and magnetic resonance imaging, with the added benefit of being feasible at the bedside or in the operating room. Precautions have to be taken during TEE to avoid an abrupt rise in blood pressure. Aortic intramural hematoma is often a precursor of classic dissection or accompanies it and can also be diagnosed by TEE, although the diagnosis is frequently difficult.

Assessment and treatment of chest pain.

Chest pain is one of the main reasons for emergency admission to hospital in the UK. Jackie Tough examines the causes and treatment of chest pain and offers a structured system for taking the patient's history.

[Usefulness and safety of the early rehabilitation program for patients with acute aortic dissection].

The usefulness and safety of the early rehabilitation program (2- and 3-week courses) were validated for patients with acute aortic dissection. This program undergone by 42 consecutive patients between 1996 and 1997 was compared to the conventional program undergone by 66 patients between 1993 and 1995, using the prognosis and complications for elderly patients. Mortality rate and morbidity rate were not significantly different between the early and conventional programs. The incidence of intensive care unit (ICU) syndrome in elderly patients was 16% (3 of 19 cases) vs 50% (15 of 30 cases), respectively (p < 0.05). The duration of hospital stays was 26 +/- 7 vs 37 +/- 13 days, respectively (p < 0.05). The early rehabilitation program for patients with acute aortic dissection was safe and useful to prevent complications in elderly patients, and was cost effective.

[Coronary assessment and surgery for aortic aneurysm: systematic coronarography]. / Bilan coronarien et chirurgie pour anévrysme aortique: coronarographie systématique.

Epidemiologic studies have shown that patients with peripheral vascular disease have a high risk of coronary heart disease. The vascular surgeon must therefore search for latent lesions which might lead to decompensation during or after the surgical procedure. Coronarography can be proposed in symptomatic patients or those with a past history of coronary heart disease, but such an indication is questionable for asymptomatic patients. Non-invasive exploration could theoretically provide information of the coronary risk but are not always possible or reliable in patients with peripheral vascular disease. A practical attitude can be established by analyzing the lesions on the basis of large international series. Cardiac exploration evaluating ischaemia can be proposed after the initial work-up to establish management strategy for intermediary cases.

Transesophageal echocardiography and magnetic resonance imaging for the assessment of saccular aneurysm of the transverse thoracic aorta.

We report a patient who initially presented with hoarseness and was admitted to our hospital with chest pain, caused by a saccular aneurysm of the thoracic aortic arch. The initial diagnosis was made by cross-sectional echocardiography, the extension and morphology of the saccular aneurysm being detected by transesophageal echocardiography. Magnetic resonance imaging confirmed the measurements of the aneurysm and clearly showed the anatomic relation with surrounding structures and arch vessels. The patient refused operation and died during in-hospital stay. A rupture of the thoracic aneurysm was the cause of death.

Concomitant abdominal aortic aneurysm and colorectal cancer: a decision analysis approach to a therapeutic dilemma.

The therapeutic approach to a patient with concomitant abdominal aortic aneurysm and colorectal carcinoma is not clear. Decision analysis helps clarify decision options and quantify therapeutic outcomes. Variables used in decision analysis include life expectancy after resection for colorectal cancer and abdominal aortic aneurysm, rupture rate of abdominal aortic aneurysm, complications of colorectal The results support the concept that the symptomatic lesion should be treated first. When both lesions are asymptomatic and the aneurysm is 4-5 cm in diameter, it should be resected first, if the colorectal cancer has a less than 5% chance of obstruction or perforation, as is found in noncircumferential lesions. When the aneurysm is greater than 5 cm, it should be resected first if the cancer has a less than 22% chance of obstructing or perforating, as with circumferential lesions. Simultaneous resection should be considered for patients with aneurysms greater than 5 cm and cancers with a greater than 75-80% chance of obstruction or perforation, provided the dual procedures can be performed with a less than 10% operative mortality and less than 50% complication rate.

Safety of simultaneous aortic reconstruction and renal transplantation.

Patients with aortic disease and end-stage renal failure who require both aortic reconstruction and renal transplantation (simultaneously or staged) pose a formidable clinical challenge. Traditionally, the performance of either one of these procedures has been viewed as a relative contraindication to the performance of the other. From 1978 to 1989, eight patients were referred to us with the combination of aortic disease and end-stage renal failure. Seven had aneurysmal disease and one had aorto-iliac occlusive disease. Five patients presented with their diseases sequentially and had two sequential operations, with a mean interval of 4 years between procedures. Three patients presented with their diseases simultaneously and underwent simultaneous aortic reconstruction and living related renal transplantation. All patients were followed up for a mean interval of 4.5 years. By life-table analysis, the 5-year renal graft survival was 100%, the primary aortic graft patency was 82%, and the secondary aortic graft patency was 100%. The only death in this series occurred 11 years after aortic reconstruction and 4 months after a renal transplantation and was due to overwhelming cytomegalovirus sepsis. There were no significant differences between the simultaneous and staged groups in terms of operative mortality, postoperative complications, transplant function, or aortic graft patency. From this experience, we conclude that: (1) patients who present simultaneously with aortic disease and end-stage renal failure can safely undergo simultaneous aortic reconstruction and renal transplantation; (2) patients who present with these two diseases sequentially can undergo a second reconstructive procedure with very low operative morbidity and mortality rates; (3) when these two procedures have been performed sequentially, the second procedure has not significantly altered the 30-day or 5-year results of the first procedure; and (4) the 30-day and 5-year results of each procedure have been excellent regardless of the temporal sequence in which they were performed.