Natural history and management of Kommerell's diverticulum in a single tertiary referral center.

OBJECTIVE: The Kommerell diverticulum (KD) is an extremely rare developmental abnormality of the aorta related to an aberrant subclavian artery (ASCA). The objective of our study was to review the natural history of KD and ASCA using our single-center experience in diagnosing and managing KD and ASCA. METHODS: A retrospective review of the Yale radiological database from January 1999 to December 2016 was performed. Only patients with KD/ASCA and a computed tomography (CT) scan of the chest were selected for review. The primary goal was to examine the natural history of KD and ASCA and the secondary goals were to review the management and outcomes of those patients treated for KD and ASCA. RESULTS: There were 75 patients with KD/ASCA identified, with a mean age of 63 ± 19 years; 49 were female (65%). On CT scans, left- and right-sided aortas were present in 47 (63%) and 28 (37%) patients. A right ASCA or a left ASCA were present in 47 (63%) and 28 (37%) patients. Six patients were symptomatic on presentation. Symptoms included dysphagia, chest or back pain, and emboli to the fingers. The mean KD diameter was 21.8 ± 6.0 mm and the distance to the opposite aortic wall (DAW) was 48.3 ± 10.8 mm. Sixty-six patients were followed for a mean of 31.7 ± 32.5 months. One patient ruptured without repair. Nine patients underwent operative intervention, including eight open and one endovascular repair. Complications from operative intervention included ischemic stroke with hemorrhagic transformation, deep vein thrombosis and pneumonia. The mean growth rate for KD and DAW was 1.45 ± 0.39 mm/year and 2.29 ± 0.47 mm/year, respectively. On multivariable regression analysis, hypertension was a predictor of growth of DAW (P = .03). CONCLUSIONS: KD is uncommon and shows a female predominance. The diverticulum grows, albeit slowly (KD and DAW growth rates of 1.45 ± 0.39 mm/year and 2.29 ± 0.47 mm/year). Most patients are asymptomatic, but dysphagia, chest/back pain, and distal emboli may occur. Rupture is rare. Symptomatic patients should be operated. Asymptomatic patients can be followed with serial CT scans.

Increased mortality in octogenarians treated for lifestyle limiting claudication.

OBJECTIVE: Treatment for lifestyle limiting claudication (LLC) that is due to infra-inguinal peripheral artery disease relies on either bypass, angioplasty, and/or stenting. Given the enthusiasm and shift toward more endovascular therapy for treatment of LLC, we sought to analyze whether octogenarians benefit from infra-inguinal interventions in the same manner as their younger counterparts. METHODS: We identified all patients admitted for elective treatment of LLC from the Nationwide Inpatient Sample from 2003 to 2012, who received open surgical or endovascular intervention for infra-inguinal peripheral arterial disease. These patients were divided into two groups including those between the ages 60-80 years (younger cohort) and those older than 80 years (octogenarians). Primary end-points included morbidity and mortality and the secondary end-points were length of hospital stay (LOS) and disposition after dismissal. RESULTS: Among 59,323 discharges identified in the dataset, 34,658 (58%) were males. There were 50,323 (85%) patients in the younger cohort and 9,000 (15%) octogenarians. The mean age was 69.9 ± 5.7 years and 84.2 ± 3.0 years for the younger cohort and octogenarians, respectively. The mean Charlson comorbidity index (CCI) was higher in our younger cohort (2.1 ± 1.1, P < 0.001). Octogenarians mainly treated with open surgery prior to 2004 are now treated endovascularly and this trend has remained stable. The younger cohort's treatment modality has fluctuated through the study period and most recently is treated mainly with open surgery. The rate of acute kidney injury, exacerbation of congestive heart failure and mortality was higher in octogenarians (P < 0.001). The rate of infectious wound complications was higher in the younger cohort (P < 0.05). Octogenarians have longer LOS and are dismissed in higher percentage to a skilled nursing facility (P < 0.001). On binary logistic regression analysis, age over 80 years, female sex, higher CCI and having an open as opposed to an endovascular procedure are independent predictors of in-hospital mortality. CONCLUSIONS: Although endovascular techniques seem to dominate the care for octogenarians with LLC, the overall morbidity and mortality rates are significantly higher in this patient population. Other options such as medical management and/or supervised exercise therapy should be explored in this patient group.

Thoracic aortic aneurysm: unlocking the "silent killer" secrets.

Thoracic aortic aneurysm (TAA) is an increasingly recognized condition that is often diagnosed incidentally. This review discusses ten of the most relevant epidemiological and clinical secrets of this disease; (1) the difference in pathogenesis between ascending and descending TAAs. TAAs at these two sites act as different diseases, which is related to the different embryologic origins of the ascending and descending aorta. (2) The familial pattern and genetics of thoracic aneurysms. Syndromic TAAs only explain 5% of the pattern of inheritance. (3) The effect of female sex on TAA growth and outcome. Females have been found to have worse outcomes compared to males. (4) Guilt by Association. TAAs are associated with abdominal aortic aneurysms, intracranial aneurysms, bicuspid aortic valve, and inflammatory disorders. (5) Natural history of TAAs. Important findings have been made regarding the expansion rate (in relation to familial pattern, location and size), and also regarding the risk of rupture or dissection. (6) The aortic size paradox. Size only is not a sufficient predictor of risk of dissection. (7) Biomarker void. Although many serum biomarkers have been studied, imaging remains the only reliable method for diagnosis and follow-up. (8) Indications for repair. Decisions are made depending on symptoms, location, size, and familial patterns. (9) Types of repair. Both open and endovascular repair options are available for certain TAAs. (10) Medical treatment. The efficacy of prescribing beta blockers, angiotensin converting enzyme inhibitors or angiotensin receptor blockers remains dubious.

Decision-making algorithm for ascending aortic aneurysm: Effectiveness in clinical application?

OBJECTIVE: The risk of rupture and dissection in ascending thoracic aortic aneurysms increases as the aortic diameter exceeds 5 cm. This study evaluates the clinical effectiveness of a specific algorithm based on size and symptoms for preemptive surgery to prevent complications. METHODS: A total of 781 patients with nondissecting ascending thoracic aortic aneurysms who presented electively for evaluation to our institution from 2011 to 2017 were triaged to surgery (n = 607, 77%) or medical observation (n = 181, 24%) based on a specific algorithm: surgery for large (>5 cm) or symptomatic aneurysms. A total of 309 of 781 patients did not undergo surgery. Of these, 128 (16%) had been triaged to prompt repair but did not undergo surgery for a variety of reasons ("surgery noncompliant and overwhelming comorbidities" group). Another 181 patients (24%) were triaged to medical management ("medical" group). RESULTS: In the "surgery noncompliant and overwhelming comorbidities" versus the "medical" group, mean aortic diameters were 5 ± 0.5 cm versus 4.45 ± 0.4 cm and aortic events (rupture/dissection) occurred in 17 patients (13.3%) versus 3 patients (1.7%), respectively (P < .001). Later elective surgeries (representing late compliance in the "surgery noncompliant and overwhelming comorbidities group" or onset of growth or symptoms in the "medical" group) were conducted in 21 patients (16.4%) versus 15 patients (8.3%) (P = .04), respectively. Death ensued in 20 patients (15.6%) versus 6 patients (3.3%) (P < .001), respectively. In the "surgery noncompliant and overwhelming comorbidities" group, 7 of 20 patients died of definite aortic causes compared with none in the "medical" group. CONCLUSIONS: Patients with ascending thoracic aortic aneurysms who did not follow surgical recommendations experienced substantially worse outcomes compared with medically triaged candidates. The specific algorithm based on size and symptoms functioned effectively in the clinical setting, correctly identifying both at-risk and safe patients.

A systematic review on management of nutcracker syndrome.

OBJECTIVE: Although nutcracker syndrome (NS) is rare, patients presenting with symptoms or signs and anatomic compression of the left renal vein (LRV) can be considered for intervention. Open, laparoscopic, and endovascular techniques have been developed to decrease the venous outflow obstruction of the LRV. The paucity of data regarding the management of this uncommon disease process poses a challenge for adequate recommendations of the best treatment modality. Herein, we aim to present a systematic review for the management of NS. METHODS: We used the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement standards to systematically search the electronic databases of MEDLINE from October 1982 to July 2017 for articles about the management of NS. Included were studies in English, Spanish, and German in all age groups. RESULTS: The literature search provided 249 references. After abstract and full review screening for inclusion, 17 references were analyzed. Eight (47%) described the open surgical approach. The LRV transposition was the most commonly reported technique, followed by renal autotransplantation. Seven (41.11%) described the endovascular technique of stent implantation, and two (11.7%) described the minimally invasive laparoscopic extravascular stent implantation. CONCLUSIONS: NS is a rare entity. Multiple techniques have been developed for the treatment of this condition. However, the rarity of this syndrome, the paucity of data, and the short-term follow-up of the existing evidence are the disadvantages that prevent recommendations for the best treatment strategy. Up to now, open surgical intervention, specifically LRV transposition, has been considered by some experts the mainstay for treatment of NS. The endovascular approach is gaining strength as more evidence has become available. However, the long-term patency and durability of this approach remain to be elucidated. Therefore, careful selection of patients is necessary in recommending this technique.

Height alone, rather than body surface area, suffices for risk estimation in ascending aortic aneurysm.

BACKGROUND: In international guidelines, risk estimation for thoracic ascending aortic aneurysm (TAAA) is based on aortic diameter. We previously introduced the aortic size index (ASI), defined as aortic size/body surface area (BSA), as a predictor of aortic dissection, rupture, and death. However, weight might not contribute substantially to aortic size and growth. We seek to evaluate the height-based aortic height index (AHI) versus ASI for risk estimation and revisit our natural history calculations. METHODS: Aortic diameters and long-term complications of 780 patients with TAAA were analyzed. Growth rate estimates, yearly complication rates, and survival were assessed. Risk stratification was performed using regression models. The predictive value of AHI and ASI was compared. RESULTS: Patients were stratified into 4 categories of yearly risk of complications based on their ASI and AHI. ASIs (cm/m2) of ≤2.05, 2.08 to 2.95, 3.00 to 3.95 and ≥4, and AHIs (cm/m) of ≤2.43, 2.44 to 3.17, 3.21 to 4.06, and ≥4.1 were associated with a 4%, 7%, 12%, and 18% average yearly risk of complications, respectively. Five-year complication-free survival was progressively worse with increasing ASI and AHI. Both ASI and AHI were shown to be significant predictors of complications (P < .05). AHI categories 3.05 to 3.69, 3.70 to 4.34, and ≥4.35 cm/m were associated with a significantly increased risk of complications (P < .05). The overall fit of the model using AHI was modestly superior according to the concordance statistic. CONCLUSIONS: Compared with indices including weight, the simpler height-based ratio (excluding weight and BSA calculations) yields satisfactory results for evaluating the risk of natural complications in patients with TAAA.

Natural history of aortic root aneurysms in Marfan syndrome.

BACKGROUND: Cardiovascular complications account for a significant proportion of the shortened lifespan of Marfan syndrome (MFS) patients, with aortic dissection being the most dreadful complication. The aortic root dilates initially in MFS patients, and given its important hemodynamic role, this can lead to aortic regurgitation and poses a substantial risk of aortic dissection. This study seeks to evaluate the natural history of aortic root aneurysms in MFS patients, with a focus on growth rates and correlation of root diameter with the risk of developing aortic complications. METHODS: Seventy-eight patients confirmed to have MFS and aortic root dilatation were retrospectively reviewed, and their aortic root diameters serially analyzed. Annual growth rate estimates and yearly rates of adverse events were computed and correlated with aortic diameter. RESULTS: The mean annual growth rate of the aortic root was estimated to be 0.26±0.05 cm/year (range 0.13 to 0.35 cm). Larger aneurysms grew faster, reaching up to 0.46 cm/year for aneurysms >6 cm. Mean age at onset of aortic dissection was 36±4 years. Annual rates of adverse events (rupture, dissection and death) were obtained using a logistic regression model at sizes 3.5, 4, 4.5, 5, 5.5 and 6 cm. A sharp increase of 23% in the probability of the risk of complications at diameters 5.5 to 6 cm was recognized. CONCLUSIONS: Aortic root aneurysms in MFS patients tend to have a faster expansion rate compared to non-MFS individuals, with aortic root diameter having a significant impact on the yearly risk of developing aortic complications.

Medical management of aortic disease in Marfan syndrome.

Marfan syndrome (MFS) is a hereditary disorder with numerous pathophysiological effects, some specifically creating elastic dysfunction in cardiovascular organs. Aortic dilatation, dissection and rupture are major concerns in the management of MFS patients. Predilection to form aneurysms is an indication for prophylactic medical management of thoracic aortic aneurysm disease in these patients. The current guidelines describe ß-blockers as the standard of care with angiotensin receptor blockers (ARBs) emerging as an equal, if not better alternative. We elaborate current evidence for and against different medical regimens used for the medical management of MFS patients.

Precipitous Resolution of Type-A Intramural Hematoma with Medical Management in a Patient with Metastatic Stage 4 Renal Cell Carcinoma.

Intramural hematoma (IMH) is a variant form of aortic dissection characterized by involvement of the aortic media without the presence of an overt intimal flap. Surgical extirpation is the standard of care for type-A IMH in the Western world. However, a conservative approach with anti-impulse therapy has been advocated especially in Japan as a viable alternative. Here, we report a case of an elderly male patient with a history of metastatic stage 4 renal cell carcinoma who was treated with anti-impulse therapy for an acute type-A IMH. Blood pressure stabilization and continuous monitoring resulted in complete resolution of the IMH within 6 days. This report illustrates how immediate medical management in patients with acute type-A IMH who are not surgical candidates can alleviate the progression or even lead to regression as early as 1 week after initiating anti-impulse therapy.

The Effect of Blood Transfusion on Outcomes in Aortic Surgery.

The use of blood transfusion in cardiac surgery varies widely. The beneficial effects of blood products are offset by an increase in morbidity and mortality. Despite multiple studies showing an association between blood product exposure and adverse short- and long-term events, it is difficult to determine causality. Nevertheless, the implication is sufficient to warrant the search for alternative strategies to reduce the use of blood products while providing a standard of care that optimizes postoperative outcomes. Aortic surgery, in particular, is associated with an increased risk of bleeding requiring a blood transfusion. There is a paucity of evidence within aortic surgery regarding the deleterious effects of blood products. Here, we review the current evidence regarding patient outcomes after blood transfusion in cardiac surgery, with special emphasis on aortic surgery.

Enfermedad cardiaca inducida por radioterapia / Radiation induced heart disease

Resumen La enfermedad cardiaca inducida por los tratamientos para el cáncer, como la quimioterapia y radioterapia, se considera cada vez más una enfermedad prevalente, que por tanto amerita conocimiento para el seguimiento adecuado, la prevención y el tratamiento de la misma. Anteriormente, este grupo de investigación publicó el artículo ``Cardiotoxicidad inducida por la quimioterapia: desde las bases moleculares hasta la perspectiva clínica'', en la que se hace una revisión acerca de los diferentes aspectos, incluyendo diagnóstico, fisiopatología, tratamiento y pronóstico de la enfermedad cardiaca inducida por radioterapia.

Abstract The heart disease induced by cancer treatments, such as chemotherapy and radiotherapy, is increasingly being seen as a prevalent disease, and as such it needs to be taken into account for its appropriate follow-up, prevention and treatment. Previously, this research group published the review, ``Chemotherapy-induced cardiotoxicity from the molecular basis to the clinical perspective'', which included a review of the different aspects, including the pathophysiology, treatment, and prognosis of heart disease induced by radiotherapy.

Cardiotoxicidad inducida por la quimioterapia desde las bases moleculares hasta la perspectiva clínica / Chemotherapy-induced cardiotoxicity from molecular bases to clinical perspective

En la actualidad, el cáncer representa una epidemia que puede ser considerada como un problema de salud pública. Es por esto que el desarrollo farmacológico ha tomado un auge en pro de mejorar las tasas de sobrevida y remisión. No obstante, esto ha sido oscurecido por la toxicidad de estas terapias. La cardiotoxicidad es un efecto adverso conocido de la terapia oncológica, que limita en muchas ocasiones una adecuada administración de regímenes con disminución de la eficacia; por esto, el entendimiento de las bases fisiopatológicas y moleculares son esenciales para determinar medidas preventivas y terapéuticas que permitan una disminución en la incidencia de la toxicidad, sin influir en la efectividad de la terapia oncológica. A continuación se realiza una revisión de los efectos adversos de la quimioterapia en el sistema cardiaco, incluyendo desde sus bases moleculares hasta sus implicaciones clínicas.

Nowadays cancer represents an epidemic that can be considered a public health problem. That is why pharmacological development has emerged for improving survival and remission rates. Nevertheless, this has been obscured by the toxicity of these therapies. Cardiotoxicity is a known adverse event of oncologic therapy that many times limits a correct regimen of administration thus lowering efficacy; therefore, understanding pathophysiological and molecular bases is essential in order to determine preventive and therapeutical measures that can allow a reduction in the incidence of toxicity, without interfering in the efficacy of the cancer therapy. We hereby describe a review of the adverse effects of chemotherapy in the cardiac system, from molecular bases to clinical implications.