Predictive value of Cardiac Magnetic Resonance: new and old parameters in the natural history of repaired Tetralogy of Fallot.

BACKGROUND: Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients. METHODS: 130 rTOF patients who underwent CMR (2006-2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome. RESULTS: Univariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p < .001; p = .037), HF (p = .049; p = .005), composite outcome (p = .039; p = .009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p < .001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy. CONCLUSIONS: Besides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role.

Reverse Mirizzi Syndrome.

A man in his 40s presented to our Hospital with abdominal pain, jaundice, and pruritus. He had a history of Alagille Syndrome treated with cholecystojejunostomy in the neonatal period because of initial misdiagnosis of biliary atresia. Laboratory investigations showed hyperbilirubinemia (total bilirubin 1.76 mg/dL [<1.2 mg/dL]; conjugated 1.06 mg/dL [<0.3 mg/dL]) and cholestasis (GGT 78 U/L [<50 U/L]; ALP 200 U/L [<50 U/L]). Transabdominal ultrasound was limited by aerobilia due to the cholecystojejuno-anastomosis. Subsequent basal CT scan revealed an impacted stone within the patient's native common bile duct (CBD). Aerobilia in intrahepatic bile ducts and gallbladder was reported. Magnetic Resonance cholangiopancreatography confirmed the gallstone in the CBD compressing cystic duct and common hepatic duct, with dilation of the upstream bile ducts. Furthermore, the native CBD was obstructed by other gallstones. In Mirizzi syndrome, gallstones impacted in gallbladder's Hartmann's pouch or cystic duct extrinsically compress CBD. We suggest naming the present condition "Reverse Mirizzi Syndrome" (Renzulli Matteo Syndrome, RMS) because it is the exact opposite of Mirizzi syndrome.

The Influence of Gd-EOB-DTPA on T2 Signal Behavior: An Example from Clinical Routine.

In the literature, it has repeatedly been stated that the introduction of hepatospecific contrast agents in Magnetic Resonance Imaging prolongs the acquisition time due to the hepatobiliary phase, normally acquired 15-20 min after injection. Many efforts have been made to shorten the time-consuming protocols, and it was demonstrated that T2-Weighted Images (T2WI) and Diffusion-Weighted Images (DWI) acquired after Gd-EOB-DTPA show a comparable diagnostic capability to pre-contrast T2WI and DWI in the detection and characterization of hepatic tumors. Therefore, T2WI and DWI are usually acquired after the acquisition of vascular phases, in the dead time until the acquisition of the hepatobiliary phase. Unfortunately, contrast agents, especially Gd-EOB-DTPA, reduce the hydrogen nuclei's relaxation time and modify signal intensity. We report a case in which, due to these limitations of the acquisition protocol, two hemangiomas showed an inhomogeneous, low signal on T2WI and DWI that was not visible in a follow-up scan a few days later. In conclusion, when liver lesions of unknown nature must be characterized, and there is a lack of previous radiological investigations, it could be useful to acquire pre-contrast T2WI and DWI to avoid diagnostic confusion, especially in non-tertiary centers.

MRI Appearance of Focal Lesions in Liver Iron Overload.

Liver iron overload is defined as an accumulation of the chemical element Fe in the hepatic parenchyma that exceeds the normal storage. When iron accumulates, it can be toxic for the liver by producing inflammation and cell damage. This can potentially lead to cirrhosis and hepatocellular carcinoma, as well as to other liver lesions depending on the underlying condition associated to liver iron overload. The correct assessment of liver iron storage is pivotal to drive the best treatment and prevent complication. Nowadays, magnetic resonance imaging (MRI) is the best non-invasive modality to detect and quantify liver iron overload. However, due to its superparamagnetic properties, iron provides a natural source of contrast enhancement that can make challenging the differential diagnosis between different focal liver lesions (FLLs). To date, a fully comprehensive description of MRI features of liver lesions commonly found in iron-overloaded liver is lacking in the literature. Through an extensive review of the published literature, we aim to summarize the MRI signal intensity and enhancement pattern of the most common FLLs that can occur in liver iron overload.

The "Bio-Crime Model" of Cross-Border Cooperation Among Veterinary Public Health, Justice, Law Enforcements, and Customs to Tackle the Illegal Animal Trade/Bio-Terrorism and to Prevent the Spread of Zoonotic Diseases Among Human Population.

Illegal animal trade (pet, wildlife, animal products, etc.) is an example of transnational organized crime (T.O.C.) that generates a large business with huge profit margins. This criminal activity causes several negative effects on human health (zoonoses), animal health and welfare, market protection, consumer fraud and may be used as tool of agro/bio-terrorism. Illegal animal trade can facilitate the spread of zoonoses that are defined as diseases and infections that are transmitted by vertebrate animals to man. Humans are affected by more than 1,700 known pathogens: 60% of existing human infectious diseases are zoonotic and at least 75% of emerging infectious diseases of humans have an animal origin and 72% of zoonoses originate from wildlife or exotic animals. The Bio-Crime Project was developed in 2017 by Friuli Venezia Giulia Region (Italy) and Land Carinthia (Austria) together with other public institutions to combat illegal animal trade and to reduce the risk of disease transmission from animals to humans. Project partners agreed that a multi-agency approach was required to tackle the illegal animal trade that was high value, easy to undertake and transnational crime. The Bio-crime model of cross-border cooperation introduces the novel approach of replicating the cooperative framework given by the triad of Veterinary Public Health, Justice and Law Enforcements/Customs across borders using the International Police and Custom Cooperation Centres (IPCCCs) as a connection link among public entities of the neighbor countries. This model has been recognized as a best practice at European level because it can be easily replicated and scaled up without any supplementary cost for Member States.