Patent foramen ovale and ischemic stroke: more shadows than lights? What the internist should know

The foramen ovale (FO) is an anatomical structure normally present in the fetus, which can persist in up to 25%-30% of the general population after birth. Normally it does not cause any harm but it has been associated with different pathological conditions; the more frequently encountered in clinical practice is an ischemic stroke or transient ischemic attack without any recognizable cause. However, a causal link between the cerebrovascular event and a right to left shunt through the FO is hard to demonstrate in most cases and which is the best treatment for secondary prevention is still a matter of debate with antithrombotic therapy or transcatheter closure of the FO as possible options. No definitive evidence of which is the most effective treatment is actually available so that a careful evaluation of each single patient is mandatory in order to select who will probably benefit the most from the interventional treatment as opposed to who will reasonably do well with medical therapy alone

Chronicle of a death foretold. It is time for echocardiographic screening in young athletes

Abstract Background The novel "Chronicle of a death foretold" by Gabriel Garcia Marquez is a story of a sudden death which could have been prevented. In 1976, within the University of Maryland basketball program and only 8 weeks apart, two athletes died suddenly during physical exertion. They were affected by hypertrophic cardiomyopathy and Marfan syndrome and in both cases an echocardiogram would have prevented the tragic epilogue. This coincidence drew everyone's attention and experts' interest on sudden death in sports. Methods and results Even in recent Italian history, unexpected deaths continue to affect athletes but surprisingly any real knowledge regarding the numbers and the impact of those tragedies must take medical literature and non-medical press into consideration. Herein we report the clinical case of a 13-year-old patient with a bicuspid aortic valve, whose mother was alarmed by the news of a young boy who died because of an anomalous origin of coronary artery (AOCA) which had not been diagnosed at transthoracic echocardiography (TTE). Her obstinacy induced the physicians to repeat TTE and led to the same diagnosis in her son: actually, his right coronary artery originated from the opposite sinus of Valsalva. The suspicion was confirmed by coronary CT scan and, thanks to appropriate therapy, the boy now fares well. Conclusions AOCA is the second most common cause of sudden death in young athletes. Although AOCA is often undetectable at ECG, TTE increases sensitivity of preparticipation screening. It could therefore allow us to avoid such coincidences and prevent sudden juvenile death

Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study

: Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: -0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (-0,14 ± 1.24 vs. -0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population

Gastrointestinal presentation of kawasaki disease: A red flag for severe disease?

Background Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology and the main cause of acquired heart disease among children in the developed world. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease. Objective To evaluate whether presenting abdominal manifestations identify a group at major risk for Intravenous immunoglobulin (IVIG)-resistance and coronary lesions. Methods Retrospective study of KD patients diagnosed between 2000 and 2015 in 13 pediatric units in Italy. Patients were divided into 2 groups according to the presence or absence of abdominal manifestations at onset. We compared their demographic and clinical data, IVIG-responsiveness, coronary ectasia/aneurysms, laboratory findings from the acute and subacute phases. Results 302 patients (181 boys) were enrolled: 106 patients with, and 196 patients without presenting abdominal features. Seasonality was different between the groups (p = 0.034). Patients with abdominal manifestations were younger (p = 0.006) and more frequently underwent delayed treatment (p = 0.014). In the acute phase, patients with abdominal presentation had higher platelet counts (PLT) (p = 0.042) and lower albuminemia (p = 0.009), while, in the subacute phase, they had higher white blood cell counts (WBC) and PLT (p = 0.002 and p < 0.005, respectively) and lower red blood cell counts (RBC) and hemoglobin (Hb) (p = 0.031 and p 0.009). Moreover, the above mentioned group was more likely to be IVIG-resistant (p < 0.005) and have coronary aneurysms (p = 0.007). In the multivariate analysis, presenting abdominal manifestations, age younger than 6 months, IVIG- resistance, delayed treatment and albumin concentration in the acute phase were independent risk factors for coronary aneurysms (respectively p<0.005, <0.005, = 0.005 and 0.009). Conclusions This is the first multicenter report demonstrating that presenting gastrointestinal features in KD identify patients at higher risk for IVIG-resistance and for the development of coronary aneurysms in a predominantly Caucasian population

Tricky case of Takayasu arteritis in a young child presenting with heart failure and femoral pulses

Tricky case of Takayasu arteritis in a young child presenting with heart failure and femoral\ua0pulse

First-in-human, off-label use of BeGraft® stenting of non-conduit, large right ventricular outflow tract for transcatheter valve landing zone preparation

Transcatheter implantation of pulmonary valve has emerged as a reliable approach in congenital heart patients presenting with chronic right ventricular volume or pressure overload after primary repair. Initial experience was limited by relatively narrow range of working diameter of transcatheter valves. Nowadays, improved technology allows extending this option to patient with large right ventricular outflow tract or conduit. A stable landing zone is of paramount importance before considering valve implantation. We present two cases of right ventricular outflow tract pre-stenting using the BeGraft® stent, which may become an interesting add to our tool kit in the preparation of valve landing zone

Hypertrophic Cardiomyopathy with Biventricular Involvement and Coronary Anomaly: A Case Report

Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) involvement has also been reported, though still not extensively characterized. We present a case of biventricular HCM with significant RV involvement in the absence of a left intraventricular gradient: RV outflow tract gradient due to hypertrophy and near obliteration of the RV cavity. Significant RV hypertrophy may cause reduced RV diastolic filling and/or RV outflow obstruction, with potentially increased incidence of symptoms of heart failure, arrhythmias, and pulmonary thromboembolism. The optimal treatment for these patients is unclear. Our patient underwent complete treatment and elimination of right ventricular obstruction, resulting in improved symptoms and a significant reduction in postoperative gradients. Direct relief of outflow tract obstruction can be achieved with low morbidity and good intermediate- to long-term results. Conventional surgery may provide significant symptomatic improvement and should thus be considered in the setting of HCM with outflow obstruction

Aortopulmonary Collateral Artery from the Proximal Ascending Aorta: A Rare Anatomical Finding

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries is a rare congenital heart lesion in which pulmonary blood supply may arise from different segments of the aorta. We report an unusual case of a newborn with a major collateral artery originating from the proximal ascending aorta. Successful reparative surgery was undertaken

Tetralogy of Fallot whit a "contralateral" ductus arteriosus

Two neonates were taken shortly after birth to our unit with a prenatal diagnosis of [S,D,S] Tetralogy of Fallot with pulmonary atresia and "unusual" aorta to pulmonary connection. The echocardiogram confirmed the main diagnosis showing: a left aortic arch with a vascular connection between the right innominate artery and the origin of the right pulmonary artery in patient A; and right aortic arch with a vascular connection between the left innominate artery and the origin of the left pulmonary artery in patient B