Surgical planning aided with 3D technologies for management of complex paracardiac tumors.

BACKGROUND: Accurate diagnosis and treatment of complex cardiac tumors poses challenges, particularly when surgical resection is considered. 3D reconstruction and printing appear as a novel approach to allow heart teams for optimal surgical and post operative care. METHODS: We report two patients with uncommon masses including a cardiac angiosarcoma (CAS) and a IgG4-related disease (IgG4-RD) with exclusive cardiac involvement. In both cases, three-dimensional (3D) reconstruction and 3D-printed models were utilized to aid the surgical team achieve optimal pre-operative planning. Both patients underwent ECG-gated cardiac computed tomography angiography (CCTA) imaging and, due to the complex anatomy of the masses, their large dimensions, proximity to vital cardiac and vascular structures, and unclear etiology, computational and 3D-printed models were created for surgical planning. An exploratory literature review of studies using 3D-printed models in surgical planning was performed. RESULTS: In case 1 (CAS), due to the size and extension of the mass to the right ventricular free wall, surgical intervention was not considered curative and, during thoracotomy, an open biopsy confirmed the imaging suspicion of CAS which guided the initiation of optimal medical treatment with chemotherapy and, after clear tumor retraction, the patient underwent a second surgical intervention, and during the 18 months of follow-up showed no signs of recurrence. In Case 2 (IgG4-RD), the patient underwent uncomplicated total surgical resection; this allowed directed treatment and, at 12 months follow-up, there are no signs of recurrence. Computational and 3D-printed models were used to plan the surgery and to confirm the findings. Limited studies have explored the use of 3D printing in the surgical planning of tumors. CONCLUSIONS: We present two patients with uncommon cardiac tumors, highlighting the significant value of 3D models in the anatomical characterization and assessment of their extension. These models may be essential in surgical planning for complex cardiovascular cases and could provide more information than conventional imaging modalities. Further studies are needed to demonstrate the impact of 3D technologies in studying cardiac tumors.

Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography for Diagnosis and Treatment of Chylopericardium After Cardiac Transplantation.

Chylopericardium is a rare complication after cardiac transplantation. We report a case of a 69-year-old woman with persistent chylopericardium after a heart transplantation due to Chagas disease. Failure of conservative treatment led to dynamic contrast-enhanced magnetic resonance lymphangiography and percutaneous radiologic intervention of the lymphatic leakage and symptoms resolution.

Severe Fontan-Associated Liver Disease and Its Association With Mortality.

Background Data are rare about the incidence of severe Fontan-associated liver disease (FALD) and its association with mortality. We sought to: (1) estimate the probability of developing severe FALD in patients who undergo the Fontan procedure (Fontan patients), compared with severe liver complications in patients with a ventricular septal defect; (2) assess the severe FALD-mortality association; and (3) identify risk factors for developing severe FALD. Methods and Results Using the Quebec Congenital Heart Disease database, a total of 512 Fontan patients and 10 232 patients with a ventricular septal defect were identified. Kaplan-Meier curves demonstrated significantly higher cumulative risk of severe FALD in Fontan patients (11.95% and 52.24% at 10 and 35 years, respectively), than the risk of severe liver complications in patients with a ventricular septal defect (0.50% and 2.75%, respectively). At 5 years, the cumulative risk of death was 12.60% in patients with severe FALD versus 3.70% in Fontan patients without FALD (log-rank P=0.0171). Cox proportional hazard models identified significant associations between the development of severe FALD and congestive heart failure and supraventricular tachycardia, with hazard ratios (HRs) of 2.36 (95% CI, 1.38-4.02) and 2.45 (95% CI, 1.37-4.39), respectively. More recent Fontan completion was related to reduced risks of severe FALD, with an HR of 0.95 (95% CI, 0.93-0.97) for each more recent year. Conclusions This large-scale population-based study documents that severe FALD in Fontan patients was associated with a >3-fold increase in mortality. The risk of FALD is time-dependent and can reach >50% by 35 years after the Fontan operation. Conditions promoting poor Fontan hemodynamics were associated with severe FALD development.