Experiences of parents of children with hypoplastic left heart syndrome during their treatment: a qualitative study.

AIM: To investigate the opinion and personal experience of parents of children born with Hypoplastic Left Heart Syndrome and what advice they would give to other parents who have to decide between treatment options. METHODS: We conducted a qualitative, descriptive and retrospective study by means of a survey directed to parents of children born with Hypoplastic Left Heart Syndrome in a tertiary hospital in Buenos Aires (Argentina). Their answers and data regarding medical procedures were analysed. RESULTS: Parents of thirteen out of sixteen patients with Hypoplastic Left Heart Syndrome were surveyed. Norwood surgery had been performed in all the patients, many had received other procedures, and five had died. In relation to the decision-making process, sixty-one percent of parents would recommend other parents to remain at peace after having done everything possible and 54% would suggest to not feel guilt despite the final result. None of the parents would recommend rejecting surgical treatment and choosing comfort care. CONCLUSION: The majority of parents of children with Hypoplastic Left Heart Syndrome would recommend continuing with the therapeutic effort in order to feel at peace and reduce feelings of guilt.

Lymphatic Decompression Concomitant With Fontan/Kreutzer Procedure: Early Experience.

OBJECTIVE: To present a strategy for identifying patients at risk of lymphatic failure in the setting of planned Fontan/Kreutzer completion, allowing a tailored surgical approach. METHODS: Since January 2017, clinical evaluation before performance of the Fontan/Kreutzer procedure included T2-weighted magnetic resonance imaging (MRI) lymphangiography. Thoracic lymphatic abnormalities were categorized using a scale of I to IV according to progression of severity. Patients with severe lymphatic abnormalities (types III and IV) underwent Fontan/Kreutzer with lymphatic decompression via connection of the left jugular-subclavian junction containing the thoracic duct to the systemic atrium (group A). RESULTS: Thirteen patients were enrolled. Magnetic resonance imaging showed type I abnormalities in four cases (30.7%), II in four (30.7%), III in two (15.3%), and IV in three (23.3%). Patients in types III and IV underwent a Fontan/Kreutzer with lymphatic decompression (group A, n = 5), while patients in types I and II underwent a fenestrated extracardiac Fontan/Kreutzer procedure without lymphatic decompression (group B, n = 8). Preoperatively, there were no differences in age, weight, ventricular dominance (right vs left), superior vena cava pressure, incidence of chylothorax after previous superior cavopulmonary anastomosis (Glenn), or need for concomitant procedures at Fontan/Kreutzer completion. There were no differences in procedural times between the groups, nor were there differences in mortalities and Fontan/Kreutzer takedowns. There were no statistically significant differences in early and late morbidity between the two groups with the exception of total volume of effusions output postoperatively. At median follow-up of 18 months (range, 4-28 months), all patients in group A are in New York Heart Association class 1 with no differences between groups in arterial oxygen saturation. CONCLUSIONS: Lymphatic decompression during Fontan/Kreutzer procedure was successfully performed in patients identified by MRI as predisposed to lymphatic failure. A larger cohort of patients and longer follow-up are required to determine the efficacy of this approach in preventing early- and long-term Fontan/Kreutzer failure.

[Extracorporeal membrane oxygenation and plasmapheresis in the treatment of severe pulmonary hemorrhage secondary to nodose polyarteritis]. / Oxigenación por membrana extracorpórea y plasmaféresis en un paciente con hemorragia pulmonar grave secundaria a poliarteritis nodosa.

We report to simultaneous use of extracorporeal life support (ECLS), plasmapheresis, hemodiafiltration and exogenous surfactant for the treatment of pulmonary hemorrhage, arterial hypertension, and renal failure secondary to nodose polyarteritis (NPA) in a 23-month-old patient. NPA is an autoimmune disease that affects small -and medium- caliber muscular arteries. Hypoxemia refractory to treatment with conventional and high frequency oscillatory ventilation was supported by ECMO while awaiting control of the autoimmune disease through the use of plasmapheresis and immunosuppressive treatment. Although the combination of ECLS with plasmapheresis is rare, it has been described during the management of cases of organ transplant, intoxication, cardiac failure, and sepsis. There are only two previous reports describing the use of this combined therapy for the treatment of pulmonary hemorrhage secondary to autoimmune vasculitis in childhood.

Oxigenación por membrana extracorpórea y plasmaféresis en un paciente con hemorragia pulmonar grave secundaria a poliarteritis nodosa / Extracorporeal membrane oxygenation and plasmapheresis in the treatment of severe pulmonary hemorrhage secondary to nodose polyarteritis

Se comunica el caso de un paciente de 23 meses de edad, con hemorragia pulmonar, hipoxemia refractaria, hipertensión arterial y falla renal secundarias a poliarteritis nodosa (PN) que ha requerido asistencia simultánea con soporte cardiopulmonar extracorpóreo (ECMO), plasmaféresis, hemodiafiltración y surfactante exógeno. La PN es una vasculitis autoinmunitaria que afecta los vasos arteriales de pequeño y mediano calibres. La hipoxemia, refractaria al tratamiento con ventilación mecánica convencional y de alta frecuencia oscilatoria, fue sostenida con ECMO mientras se controló la enfermedad autoinmunitaria mediante plasmaféresis y tratamiento inmunosupresor. La combinación de ECMO con plasmaféresis es infrecuente, pero ha sido descripta en relación al trasplante de órganos, intoxicaciones, fallo cardíaco y sepsis. Existen dos casos descriptos en la bibliografía sobre el uso combinado de estas terapéuticas para el tratamiento de hemorragia pulmonar secundaria a vasculitis autoinmunitaria en niños.

We report to simultaneous use of extracorporeal life support (ECLS), plasmapheresis, hemodiafiltration and exogenous surfactant for the treatment of pulmonary hemorrhage, arterial hypertension, and renal failure secondary to nodose polyarteritis (NPA) in a 23-month-old patient. NPA is an autoimmune disease that affects small -and medium- caliber muscular arteries. Hypoxemia refractory to treatment with conventional and high frequency oscillatory ventilation was supported by ECMO while awaiting control of the autoimmune disease through the use of plasmapheresis and immunosuppressive treatment. Although the combination of ECLS with plasmapheresis is rare, it has been described during the management of cases of organ transplant, intoxication, cardiac failure, and sepsis. There are only two previous reports describing the use of this combined therapy for the treatment of pulmonary hemorrhage secondary to autoimmune vasculitis in childhood.

Oxigenación por membrana extracorpórea y plasmaféresis en un paciente con hemorragia pulmonar grave secundaria a poliarteritis nodosa / Extracorporeal membrane oxygenation and plasmapheresis in the treatment of severe pulmonary hemorrhage secondary to nodose polyarteritis

Se comunica el caso de un paciente de 23 meses de edad, con hemorragia pulmonar, hipoxemia refractaria, hipertensión arterial y falla renal secundarias a poliarteritis nodosa (PN) que ha requerido asistencia simultánea con soporte cardiopulmonar extracorpóreo (ECMO), plasmaféresis, hemodiafiltración y surfactante exógeno. La PN es una vasculitis autoinmunitaria que afecta los vasos arteriales de pequeño y mediano calibres. La hipoxemia, refractaria al tratamiento con ventilación mecánica convencional y de alta frecuencia oscilatoria, fue sostenida con ECMO mientras se controló la enfermedad autoinmunitaria mediante plasmaféresis y tratamiento inmunosupresor. La combinación de ECMO con plasmaféresis es infrecuente, pero ha sido descripta en relación al trasplante de órganos, intoxicaciones, fallo cardíaco y sepsis. Existen dos casos descriptos en la bibliografía sobre el uso combinado de estas terapéuticas para el tratamiento de hemorragia pulmonar secundaria a vasculitis autoinmunitaria en niños.(AU)

We report to simultaneous use of extracorporeal life support (ECLS), plasmapheresis, hemodiafiltration and exogenous surfactant for the treatment of pulmonary hemorrhage, arterial hypertension, and renal failure secondary to nodose polyarteritis (NPA) in a 23-month-old patient. NPA is an autoimmune disease that affects small -and medium- caliber muscular arteries. Hypoxemia refractory to treatment with conventional and high frequency oscillatory ventilation was supported by ECMO while awaiting control of the autoimmune disease through the use of plasmapheresis and immunosuppressive treatment. Although the combination of ECLS with plasmapheresis is rare, it has been described during the management of cases of organ transplant, intoxication, cardiac failure, and sepsis. There are only two previous reports describing the use of this combined therapy for the treatment of pulmonary hemorrhage secondary to autoimmune vasculitis in childhood.(AU)