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A prospective, observational single-center study was carried out. Pediatric patients undergoing congenital heart defect surgery were evaluated before, during, and after surgery. At each time point, sublingual microcirculation and clinical parameters were assessed, along with analytical variables. Twenty-four patients were included. All microcirculatory parameters worsened during cardiopulmonary bypass and returned to baseline values after surgery (p ≤ 0.001). In the intraoperative evaluation, body temperature correlated with perfused small vessel density (p = 0.014), proportion of perfused small vessels (p < 0.001), small vessel microvascular flow index (p = 0.003), and small vessel heterogeneity index (p < 0.002). Patients with cyanotic disease exhibited higher small vessel density (p < 0.008) and higher density of perfused small vessels (p < 0.022) at baseline, and a lower microvascular flow index (p = 0.022) and higher heterogeneity (p = 0.026) in the intraoperative phase. Children with congenital heart disease exhibited decreased vascular density and microvascular blood flow and increased heterogeneity during cardiopulmonary bypass. All these parameters returned to baseline values after surgery.

Genetics; Heritable pulmonary arterial hypertension; Pediatric pulmonary hypertension Genética; Hipertensión arterial pulmonar hereditaria; Hipertensión pulmonar pediátrica Genètica; Hipertensió arterial pulmonar hereditària; Hipertensió pulmonar pediàtrica Background: Pulmonary arterial hypertension (PAH) is a severe and rare disease with an important genetic background. The influence of genetic testing in the clinical classification of pediatric PAH is not well known and genetics could influence management and prognosis. Objectives: The aim of this work was to identify the molecular fingerprint of PH children in the REgistro de pacientes con HIpertensión Pulmonar PEDiátrica (REHIPED), and to investigate if genetics could have an impact in clinical reclassification and prognosis. Methods: We included pediatric patients with a genetic analysis from REHIPED. From 2011 onward, successive genetic techniques have been carried out. Before genetic diagnosis, patients were classified according to their clinical and hemodynamic data in five groups. After genetic analysis, the patients were reclassified. The impact of genetics in survival free of lung transplantation was estimated by Kaplan–Meier curves. Results: Ninety-eight patients were included for the analysis. Before the genetic diagnoses, there were idiopathic PAH forms in 53.1%, PAH associated with congenital heart disease in 30.6%, pulmonary veno-occlusive disease—PVOD—in 6.1%, familial PAH in 5.1%, and associated forms with multisystemic disorders—MSD—in 5.1% of the patients. Pathogenic or likely pathogenic variants were found in 44 patients (44.9%). After a genetic analysis, 28.6% of the cohort was “reclassified”, with the groups of heritable PAH, heritable PVOD, TBX4, and MSD increasing up to 18.4%, 8.2%, 4.1%, and 12.2%, respectively. The MSD forms had the worst survival rates, followed by PVOD. Conclusions: Genetic testing changed the clinical classification of a significant proportion of patients. This reclassification showed relevant prognostic implications. This project was funded by project “Bases Genético-Moleculares de la Medicina de Precisión en la Hipertensión Arterial Pulmonar”. Instituto de Salud Carlos III, Ministerio de Economía y Competitividad, Gobierno de España. Co-funded by “Fondo Europeo de Desarrollo Regional, Programa Operativo Crecimiento Inteligente 2014–2020” (Award number: PI 18/01233). A.C.-U. holds a research-training contract “Rio Hortega” (CM20/00164) from the Spanish Ministry of Science and Innovation (Instituto de Salud Carlos III). REHIPED is supported by unrestricted grants of Janssen and Ferrer.

Pulmonary percutaneous valve implantation (PPVI) is feasible with satisfactory mid-term results in patients with native right ventricular outflow tract (RVOT) and has been increasingly used instead of surgically implantable pulmonary valves. Creating a stable landing zone with a diameter less than the largest commercially available valve (previously available 29 mm and currently available 32 mm) is crucial for technical success of the procedure, limiting the number of suitable candidates for PPVI. We report the case of PPVI with a 32 mm Myval transcatheter heart valve in a patient with a large native RVOT (pre-stented with AndraStent XXL mounted on a 35 × 60 mm valve balloon catheter) lesion who had Tetralogy of Fallot surgically corrected. The post-procedural outcomes of this case were satisfactory with no complications reported during the hospital stay.

A prospective, observational single-center study was carried out. Pediatric patients undergoing congenital heart defect surgery were evaluated before, during, and after surgery. At each time point, sublingual microcirculation and clinical parameters were assessed, along with analytical variables. Twenty-four patients were included. All microcirculatory parameters worsened during cardiopulmonary bypass and returned to baseline values after surgery (p ≤ 0.001). In the intraoperative evaluation, body temperature correlated with perfused small vessel density (p = 0.014), proportion of perfused small vessels (p0.001), small vessel microvascular flow index (p = 0.003), and small vessel heterogeneity index (p0.002). Patients with cyanotic disease exhibited higher small vessel density (p0.008) and higher density of perfused small vessels (p0.022) at baseline, and a lower microvascular flow index (p = 0.022) and higher heterogeneity (p = 0.026) in the intraoperative phase. Children with congenital heart disease exhibited decreased vascular density and microvascular blood flow and increased heterogeneity during cardiopulmonary bypass. All these parameters returned to baseline values after surgery.

Sr. Editor: Durante los últimos años se está empezando a tomar conciencia del impacto adverso de la insuficiencia tricuspídea en la morbilidad (empeoramiento de la calidad de vida, ingresos hospitalarios…) y en la mortalidad, con el consiguiente incremento del intervencionismo tanto quirúrgico como percutáneo sobre dicha válvula1,2. Desde el punto de vista quirúrgico, la técnica más utilizada para el tratamiento de la valvulopatía tricúspide es la reparación mediante anuloplastia para intentar reducir el tamaño del anillo y facilitar la coaptación de los velos, normalmente con anillos incompletos para respetar el sistema de conducción septal. Los resultados a corto plazo son satisfactorios en la mayoría de los casos, pero según las series hasta el 25% de los pacientes presentan insuficiencia moderada o grave a los 5 años. En general se trata de pacientes de gran complejidad, con múltiple comorbilidad y varias cirugías cardiacas previas, con el consiguiente riesgo quirúrgico, por lo que el desarrollo de las técnicas de intervencionismo percutáneo puede ser un gran aliado al respecto. En la actualidad, la mayor experiencia en el tratamiento percutáneo tricuspídeo se tiene en la disfunción protésica tricuspídea mediante procedimientos valve-in-valve, con buenos resultados. En cuanto al implante percutáneo de bioprótesis sobre anillos disfuncionantes, suelen ser...

To the Editor, Over the last few years we have become aware of the adverse impact of tricuspid regurgitation on morbidity (worse quality of life, hospital admissions…) and mortality with the corresponding increase in the number of interventions performed on the tricuspid valve both surgically and percutaneously.1,2 From the surgical point of view, the most widely used technique for the management of tricuspid valve disease is repair with an annuloplasty to reduce the size of the ring and facilitate leaflet coaptation, usually with incomplete rings, in an attempt to spare the septal conduction system. Short-term results are satisfactory in most cases but according to the series published so far, up to 25% of the patients show moderate or severe regurgitation at 5 years. Overall, we are talking about patients of great complexity, multiple comorbidities, and several prior cardiac surgeries with the corresponding surgical risk, which is why the development of percutaneous coronary intervention techniques may be a great ally. Currently the treatment of tricuspid valve dysfunction through valve-in-valve procedures is the percutaneous treatment of the tricuspid valve for which we have more and most successful experience. The percutaneous implantation of valves in dysfunctional rings are usually procedures with a series of difficulties...

Breast tumors in adolescents are very rare and mostly benign. Fibroadenomas are the most frequent, but within the extensive differential diagnosis, the phyllodes tumor must be mentioned, which accounts for about 1% of breast tumors and the diagnosis of which is very rare in patients younger than 20 years. There are no specific symptoms or radiological images to distinguish phyllodes tumor from fibroadenoma; therefore, histological examination is mandatory for diagnosis. Histology also allows the classification of phyllodes tumor into benign, borderline, or malignant types for appropriate surgical treatment: freemargin excision in benign tumors and mastectomy in the other two types. Fortunately, the majority of these tumors are benign, and treatment maximizes breast conservation with free infiltration margins surgery, given that this fact is the most important factor to prevent local recurrence. In this article, we describe a rare case of borderline cystosarcoma phyllodes in a 12-year-old girl.