Maria Vincenza Polito

Aortic dissection in patients with autosomal dominant polycystic kidney disease: a series of two cases and a review of the literature.

Aortic dissection (AD) is the most common life‐threatening disease involving the aorta. It is rarely associated with systemic disorders such as Autosomal Dominant Polycystic Kidney Disease (ADPKD), a genetic syndrome characterized by cystic degeneration of kidneys, possible presence of cysts in other organs and extra‐renal manifestations, including cardiovascular disorders. We performed a systematic literature search focused on the occurrence of AD associated with ADPKD (25 cases identified), and reported two cases from our experience. We selected data on sex, age, family history of ADPKD and/or AD, habitus, hypertension, renal function, presence of hepatic/pancreatic/splenic cysts, clinical presentation of AD, AD type according to the Stanford classification, treatment and outcome. Furthermore we compared this dataset with the data of the overall population with AD from the International Registry of Acute Aortic Dissection (IRAD). Stanford A type AD was documented in 62% of patients. As expected, the initial manifestation of AD was most commonly chest and back pain (80%). The mean age of AD occurrence appears significantly reduced in ADPKD patients compared to the general population with AD (49 ± 12 vs 62 ± 14, P < 0.001). Of note, our analysis shows a remarkably higher frequency of hypertension (90%) compared to the overall AD population (75%), although not significantly (P = 0.133). AD should be always ruled out in ADPKD subjects with chest and back pain symptoms, despite them being young and at lower risk. A careful preventive monitoring as well as an optimal blood pressure control may reduce the risk of AD and improve the outcome of these patients.

Stress-related cardiomyopathy, ventricular dysfunction, artery thrombosis: a hidden pheochromocytoma

Clinical presentation of pheochromocytoma can vary, and it can sometimes mimic other diseases. Some patients with pheochromocytoma may have atypical presentations, such as clinical features consistent with an acute coronary syndrome, that only later suggest a classical picture of stress-related cardiomyopathy. To our best knowledge, pheochromocytoma has been incidentally revealed in a few cases of catecholamine-induced cardiomyopathy and in only 1 case of peripheral arterial thrombosis. This is the first case of pheochromocytoma revealed after left ventricular dysfunction caused by stress-related cardiomyopathy associated with inferior limb artery thrombosis in a patient with a complex cardiovascular history.

A peculiar etiology of acute heart failure: adrenergic myocarditis

Pheochromocytoma can occur with a variety of cardiovascular signs and symptoms, and this tumor can also precipitate an acute heart failure associated with the typical clinical and instrumental findings of myocarditis. This peculiar etiology of acute myocarditis, known as “adrenergic myocarditis,” should be suspected when specific “red flags” of pheochromocytoma such as headache, palpitations, diaphoresis, hypertension, orthostatic hypotension, and left ventricular dysfunction suggesting Takotsubo syndrome are detected. In fact, its diagnosis allows a specific targeted therapy.

Tight Bra in a 34-Year-Old Woman: An Unusual Cause of Mondor’s Disease

Mondors disease is characterized by thrombophlebitis of the superficial veins of the breast and the chest wall. The list of causes is long. Various types of clothing, mainly tight bras and girdles, have been postulated as causes. We report a case of a 34-year-old woman who referred typical symptoms and signs of Mondors disease, without other possible risk factors, and showed the cutaneous findings of the tight bra. Therefore, after distinguishing benign causes of Mondors disease from hidden malignant causes, the clinicians should consider this clinical entity.

Effects of aortic counterpulsation in 6 cases of fulminant myocarditis

[1] Ohlow MA, Wong V, Brunelli M, von Korn H, Farah A, Memisevic N, et al. Acute coronary syndrome without critical epicardial coronary disease: prevalence, characteristics, and outcome. Am J Emerg Med 2015;33(2):150–4. [2] Biteker M. Current understanding of Kounis syndrome. Expert Rev Clin Immunol 2010;6(5):777–88. [3] Biteker M. A new classification of Kounis syndrome. Int J Cardiol 2010;145(3):553. [4] Biteker M, Duran NE, Biteker FS, Gündüz S, Gökdeniz T, Kaya H, et al. Kounis syndrome secondary to cefuroxime-axetil use in an octogenarian. J Am Geriatr Soc 2008;56(9):1757–8. [5] Biteker M, Duran NE, Biteker F, Civan HA, Gündüz S, Gökdeniz T, et al. Kounis syndrome: first series in Turkish patients. Anadolu Kardiyol Derg 2009;9(1):59–60.

Stress-induced cardiomyopathy in pheochromocytoma: the way we treat and the way we think

We thank the authors for the relevant comment to our case report; their thoughtful input gives us the opportunity to specify some concepts. First of all, as stated by modified Mayo Clinic diagnostic criteria for Tako-tsubo cardiomyopathy [1] and new diagnostic criteria proposed by Tako-tsubo Italian Network [2], once Tako-tsubo cardiomyopathy has been suspected, a diagnostic work-up to rule out pheochromocytoma should be systematically performed. Owing to its transient left ventricular dysfunction [3], the treatment of stress-related cardiomyopathy (SRC) is mainly supportive, including angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, diuretics, and β-blockers. Diuretics are indicated especially when acute heart failure occurs. β-Blockers should be used with caution in case of cardiogenic shock and only if the left ventricular outflow tract obstruction is associated [4]. In this case, an excessive dehydration has to be avoided. Because of the high catecholamine spillover in the pathophysiology of SRC, inotropic agents can have deleterious effects and are not indicated. Thus, in case of hemodynamic instability, intra-aortic balloon pump should be preferred. Stress-related cardiomyopathy, in the acute phase, is characterized by low cardiac output and low preload, so in our opinion, a pharmacologic treatment with α-adrenergic blockers that further reduce the peripheral vascular resistance do not seem helpful. Moreover, in the pathophysiology of SRC, the direct effects of catecholamines on themyocardium is related to negative inotropic effects of epinephrine via stimulation of the cardioprotective β2-adrenergic receptors–Gi signaling pathway [5], whereas α-adrenergic receptors do not seem to play a prominent role. On the other hand,α-blockade is thefirst choice for themanagement of pheochromocytoma crisis [6] and to reduce the risk of hypertensive crises in patients with biochemically active pheochromocytoma [7]. α-Blockade should be started before β-blockade because stimulation of α-adrenoceptors unopposed by β-adrenoceptor vasodilation can result in hypertensive crises [6,7]. Vasculitis is a well-described but rare autoimmune complication of pheochromocytoma, triggered by an excess of cathecolamine levels [8]. Cathecolamine-induced vascular damage is characterized by irregularities of vascular wall, stenosis or aneurysms, generally detected by angiography or computed tomography angiography [9]. Arterial caliper variation resembling vasculitis or vasospasm has been reported without histopathologic changes of intraluminal thrombosis or vasculitis, for this reason, named pseudovasculitis [10]. Isolated arterial thrombosis is not a characteristic complication of cathecolamine-induced vasculitis. In our patient, with the exception

Cardiac sarcoidosis: a challenging diagnosis

Sarcoidosis is a systemic granulomatous disease with pulmonary affection in more than 90% of cases [1]. Lymph nodes, liver, spleen, skin, and eyes are commonly involved [2]. Cardiac involvement is frequent, but rarely diagnosed and rarely the only manifestation of the disease. A 48-year-old woman presented at the emergency department because of hypotension and profuse sweating. She had no cardiovascular risk factors, except smoking and reported paucisymptomatic palpitations at rest. The electrocardiogram (ECG) showed a torsade de pointes, which was treated with synchronized cardioversion (100 J), restoring sinus rhythm. Basal EKG showed QS complex in V1–V3 precordial leads with poor R wave progression from V1 to V6 precordial leads, sporadic ventricular extrasystoles, and left anterior fascicular block (Fig. 1). In transthoracic echocardiography (TTE), a mildly reduced left ventricular (LV) systolic function (LV ejection fraction 50%) with a mildly increased LV end diastolic volume (LVEDV/BSA 80 mL/m2) and akinesia with thinning of the basal and mid-ventricular segments of the interventricular septum (IVS) were documented. Transthoracic two and three-dimensional echocardiography (3DE) confirmed a severe thinning of the interventricular septum (IVS 4 mm). Wall motion analysis by 2D speckle tracking (ST) showed a reduced global longitudinal peak-systolic strain (GLPSS − 18%). Regional longitudinal peak-systolic strain was significantly impaired in the mid-basal segments of the inferior wall, and the inferoseptal and the anteroseptal wall. In the same segments, the post-systolic index was abnormal and time to peak longitudinal strain was longer (Fig. 2). Hypothesizing a previous asymptomatic myocardial infarction, coronary angiography was performed that ruled out coronary heart disease. Thus, cardiac magnetic resonance imaging (cMRI) was performed showing mildly depressed LV systolic function as observed by TTE. T2-weighted images showed no edema, whereas post-contrast T1-weighted images documented transmural late gadolinium enhancement (LGE) in the basal segment of the IVS and the right ventricular free wall (Fig. 3). Endomyocardial biopsies (EMB) were performed to evaluate the underlying etiology of cardiac changes. As shown in Fig. 4, Masson’s Trichrome and Giemsa stainings reveal numerous granulomas consisting of multinucleate giant cells (see also immunohistochemical MCHII staining) and CD3+ T cells in the absence of eosinophils (Giemsa stain) reflecting severe cardiac sarcoidosis. However, high-resolution computed tomography (CT) as well as lung function test did not show any additional pathologic findings (Fig. 5). Blood examinations revealed anemia (Hb 9.2—v.n. > 14 g/dL), an increase in LDH (520—v.n. < 300 mL/mL), and elevated Ca++ values (13.2—v.n. < 10.5 mg/dL), as well as an increase of angiotensin-converting enzyme (ACE) (150—v.n < 82 U/L), while soluble interleukin-2 receptor (sIL-2R) was normal (394—v.n < 623 U/mL). These laboratory and diagnostic findings identified myocardial sarcoidosis and prednisone * Maria Vincenza Polito mvpolito@hotmail.it

Prognostic value of clinical, echocardiographic and angiographic indicators in patients with large anterior ST-segment elevation myocardial infarction as a first acute coronary event

Background The risk of death in patients affected by ST-elevation segment myocardial infarction (STEMI) is well known, but more data are required to define the in-hospital mortality in special subsets. We sought to assess the prognostic value of indicators in patients with large anterior STEMI as a first acute coronary event, undergoing percutaneous coronary intervention (PCI) and intra-aortic balloon pump (IABP). Methods and results We evaluated 48 consecutive large anterior STEMI patients admitted as first acute coronary event, undergoing in acute phase both PCI and IABP. Patient demographics, clinical, noninvasive and invasive findings, together with in-hospital complications, were collected. Moreover, findings obtained after a 24-month follow-up were reported. The primary endpoint was in-hospital mortality, whereas the secondary endpoints were out of hospital mortality, rehospitalization for heart failure or reinfarction, and New York Heart Association (NYHA) class at least 2 at follow-up visit. The univariate analysis showed a significant association with symptom to balloon, left anterior descending coronary artery, myocardial blush grade, and wall motion score index. Results of the multivariable analysis revealed the strongest predictive power for in-hospital mortality of proximal left anterior descending coronary artery (odds ratio: 6.9; 95% confidence interval: 1.1–67.7) and of myocardial blush grade 0–1 (odds ratio: 5.5; 95% confidence interval: 1.0–38.8). In-hospital death occurred in 13 patients (27% of total cases), whereas, at follow-up, the mean of survival was 66.7 ± 7.0%. Conclusion The patients with large anterior STEMI as a first acute coronary event, undergoing PCI and IABP, had a very high in-hospital mortality, whereas the mortality rate over the follow-up period was lower. The involvement of a large territory at risk and the ineffective treatment in terms of myocardial reperfusion were the main predictors of in-hospital mortality.

Advantages of a workbench reshaped AR1 mod catheter for right coronary angiography by right radial approach

Transradial approach in cardiac catheterization is increasing. In daily practice, coronary angiography via radial artery is usually performed by using catheters designed for femoral approach. The aim of this study was to evaluate advantages in the use of a workbench reshaped AR1 mod catheter, in terms of procedural duration time, number of catheters per procedure, fluoroscopy time, contrast agent administered volume, images quality and costs. Two hundred patients, submitted to coronary angiography via right radial artery in our institution, have been retrospectively reviewed. Patients have been divided in two groups, depending on whether a workbench reshaped Cordis Amplatz AR1 mod catheter (rAR1 mod), or catheters in their original shape (OC) have been employed. In the rAR1 mod group (100 patients) a lower number of catheters per procedure (1.07 ± 0.25 vs. 1.47 ± 1.65; p < 0.001), a more frequent right coronary selective engagement (76.76% vs. 53.12%; p < 0.001), a smaller amount of contrast agent (63.02 ± 27.77 vs. 80.85 ± 29.22 ml, p < 0.001), a reduced fluoroscopy and global procedural time (4.19 ± 2.91 vs. 5.69 ± 3.85 min, p = 0.004; and 34.58 ± 17.05 vs. 42.58 ± 17.26 min, p = 0.001, respectively) were observed. According to our experience, when right coronary angiography via right radial approach is performed, the utilization of rAR1 mod catheter correlates with multiple advantages in terms of procedural parameters.