RESUMEN
Although the terms "single ventricle" and "univentricular heart" are frequently used to describe a variety of complex congenital heart defects, in fact, nearly all hearts have two ventricles, although one of the two may be too small to be functional. A better term for these hearts would therefore be "functional single ventricle."
Asunto(s)
Atresia Tricúspide , Corazón Univentricular , Humanos , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/anomalías , Atresia Tricúspide/cirugía , Atresia Tricúspide/fisiopatología , Atresia Tricúspide/diagnóstico por imagen , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/terapia , Corazón Univentricular/genéticaRESUMEN
Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making.
Asunto(s)
Cardiopatías Congénitas , Atresia Pulmonar , Válvula Pulmonar , Atresia Tricúspide , Tabique Interventricular , Recién Nacido , Masculino , Humanos , Atresia Tricúspide/diagnóstico por imagen , Atresia Tricúspide/cirugía , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/anomalías , Arteria Pulmonar/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugíaRESUMEN
INTRODUCTION: Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia. MATERIALS AND METHODS: A retrospective observational descriptive study. The 81 patients who underwent the Fontan procedure were included. Categorical variables were presented with percentages and continuous variables with measures of central tendency according to the distribution of the data evaluated through the Shapiro-Wilk test. Sociodemographic, clinical, surgical variables, complications, and mortality were described. RESULTS: Between 2000 and 2020, 81 patients underwent the Fontan procedure: 43 (53.1%) males and a median age of 5.3 years (interquartile range 4.3-6.6). The most common diagnosis was tricuspid atresia (49.4%). The median mean pulmonary arterial pressure was 12 mmHg (interquartile range 10-15), the Nakata index 272 mm2/m2 (interquartile range 204-327), and the McGoon index (interquartile range 1.86-2.3). Seventy-two (88.9%) patients underwent extracardiac Fontan and 44 (54.3%) patients underwent fenestration. The median hospitalisation days were 19 days. The main complication was coagulopathy (19.8%), mortality in the first month between 2000 and 2010 was 8.6%, and after 2010 was 1.2%. CONCLUSION: The Fontan procedure is a palliative surgery for children with complex heart disease. According to anatomical and physiological variables, the proper choice of patients determines the short- and long-term results.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Atresia Tricúspide , Corazón Univentricular , Niño , Preescolar , Femenino , Humanos , Masculino , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/cirugía , Estudios Retrospectivos , Centros de Atención Terciaria , Resultado del Tratamiento , Atresia Tricúspide/cirugíaRESUMEN
Prolonged pleural effusion is a fairly common condition which has considerable impact on complicated and longer hospital stays after Fontan surgery. Identifying the patient population prone to have pleural effusions is still seeking for an answer. This study is to determine the variables that may predict prolonged pleural effusion according to the data of 69 patients who underwent Fontan operation between June 2018 and December 2020 and survived to date. Prolonged pleural effusion was defined as the need for a chest tube for more than 7 days. Two patient groups, with and without prolonged effusion, were compared in terms of pre-, peri-, and post-operative variables. The patients were subdivided into "high-risk" and "low-risk" groups based on the pre-operative catheterisation data. The most frequent main diagnosis was tricuspid atresia (n: 13, 19%). Among 69 patients, 28 (40%) had prolonged pleural effusion whereas 11 (16%) had effusions that lasted longer than 14 days. Ten patients among prolonged effusion group (35%) had pulmonary atresia coexistent with the main diagnosis. Fontan operation was performed in 6 patients (8.7%) over the age of 10, and 4 of these patients (67%) had prolonged pleural effusion. Among numerous variables, statistical significance between the two groups was achieved in pre-operative mean pulmonary artery pressure, post-operative albumin, C-reactive protein levels, length of hospital stay, duration of chest tube drainage, and amount of effusion per day. Early recognition and treatment strategies with routine medical protocol use remain to be the cornerstone for the management of post-operative prolonged pleural effusions after Fontan surgery.
Asunto(s)
Procedimiento de Fontan , Derrame Pleural , Atresia Tricúspide , Humanos , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Derrame Pleural/diagnóstico , Derrame Pleural/etiología , Derrame Pleural/epidemiología , Atresia Tricúspide/complicaciones , Atresia Tricúspide/cirugíaRESUMEN
Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with interrupted aortic arch. We present the diagnosis and initial surgical management of an infant with a fetal diagnosis of tricuspid atresia and common arterial trunk, with additional postnatal finding of interrupted aortic arch with interruption between the left common carotid and left subclavian artery. Due to the infant's small size, she was initially palliated with bilateral pulmonary artery bands and a ductal stent. This was followed by septation of the common arterial trunk and interrupted aortic arch repair and 4 mm right subclavian artery to main pulmonary artery shunt placement at two months of age. She was discharged home on day of life 81.
Asunto(s)
Atresia Tricúspide , Tronco Arterial Persistente , Lactante , Femenino , Humanos , Atresia Tricúspide/diagnóstico por imagen , Atresia Tricúspide/cirugía , Tronco Arterial Persistente/cirugía , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Arteria Pulmonar/anomalías , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Aorta Torácica/anomalíasRESUMEN
INTRODUCTION: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. METHODS: The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. RESULTS: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. CONCLUSIONS: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.
Asunto(s)
Procedimiento de Fontan , Puente Cardíaco Derecho , Cardiopatías Congénitas , Atresia Tricúspide , Tronco Arterial Persistente , Corazón Univentricular , Embarazo , Masculino , Humanos , Femenino , Lactante , Corazón Univentricular/cirugía , Cardiopatías Congénitas/cirugía , Puente Cardíaco Derecho/métodos , Arteria Pulmonar/cirugía , Arteria Pulmonar/anomalías , Atresia Tricúspide/cirugía , Tronco Arterial Persistente/cirugía , Estudios Retrospectivos , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/anomalías , Resultado del TratamientoRESUMEN
Isoproterenol stress test during cardiac catheterization unmasked dynamic bulbo-ventricular foramen restriction in a 5-year-old boy with bidirectional Glenn anastomosis for tricuspid atresia/transposed great arteries and unexplained syncope.
Asunto(s)
Atresia Tricúspide , Anastomosis Quirúrgica , Cateterismo Cardíaco , Preescolar , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Humanos , Isoproterenol , Masculino , Atresia Tricúspide/cirugíaRESUMEN
Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature.
Asunto(s)
Procedimiento de Fontan , Atresia Pulmonar , Válvula Pulmonar , Atresia Tricúspide , Humanos , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugía , Válvula Pulmonar/anomalías , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Atresia Tricúspide/diagnóstico por imagen , Atresia Tricúspide/cirugíaRESUMEN
Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.
Asunto(s)
Procedimiento de Fontan , Atresia Pulmonar , Válvula Pulmonar , Atresia Tricúspide , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Humanos , Atresia Pulmonar/cirugía , Válvula Pulmonar/anomalías , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Atresia Tricúspide/cirugíaRESUMEN
BACKGROUND: COVID-19 and Fontan physiology have each been associated with an elevated risk of venous thromboembolism (VTE), however little is known about the risks and potential consequences of having both. CASE PRESENTATION: A 51 year old male with tricuspid atresia status post Fontan and extracardiac Glenn shunt, atrial flutter, and sinus sick syndrome presented with phlegmasia cerulea dolens (PCD) of the left lower extremity in spite of supratherapeutic INR in the context of symptomatic COVID-10 pneumonia. He was treated with single session, catheter directed mechanical thrombectomy that was well-tolerated. CONCLUSIONS: This report of acute PCD despite therapeutic anticoagulation with a Vitamin K antagonist, managed with emergent mechanical thrombectomy, calls to attention the importance of altered flow dynamics in COVID positive patients with Fontan circulation that may compound these independent risk factors for developing deep venous thrombosis with the potential for even higher morbidity.
Asunto(s)
COVID-19 , Procedimiento de Fontan , Gangrena , Trombolisis Mecánica , Complicaciones Posoperatorias , Tromboflebitis , Atresia Tricúspide , Warfarina/uso terapéutico , Amputación Quirúrgica/métodos , Aleteo Atrial/tratamiento farmacológico , Aleteo Atrial/etiología , COVID-19/sangre , COVID-19/complicaciones , COVID-19/terapia , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/métodos , Gangrena/etiología , Gangrena/cirugía , Cardiopatías Congénitas/cirugía , Humanos , Procesamiento de Imagen Asistido por Computador/métodos , Extremidad Inferior/irrigación sanguínea , Extremidad Inferior/patología , Extremidad Inferior/cirugía , Masculino , Trombolisis Mecánica/efectos adversos , Trombolisis Mecánica/métodos , Persona de Mediana Edad , Flebografía/métodos , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/fisiopatología , Complicaciones Posoperatorias/cirugía , Síndrome del Seno Enfermo/diagnóstico , Síndrome del Seno Enfermo/etiología , Tromboflebitis/diagnóstico , Tromboflebitis/etiología , Tromboflebitis/cirugía , Tomografía Computarizada por Rayos X/métodos , Resultado del Tratamiento , Atresia Tricúspide/etiología , Atresia Tricúspide/cirugíaRESUMEN
We present the electrocardiographic findings in a 36-year-old female with tricuspid atresia with double-outlet right ventricle and malposition of great arteries who underwent classical or "atriopulmonary" Fontan procedure in childhood. Her electrocardiograms have consistently shown marked intra-atrial delay with an initial positive P wave deflection and terminal negative P deflection in all leads with progressive increase in P wave duration with time. She has had frequent episodes of intra-atrial tachycardia, atrial fibrillation and sinus and post ectopic pauses over the past few years. The findings in our patient, which have not been reported before, illustrate the atrial pathology, which is unique to Fontan physiology, particularly those with classical or atriopulmonary Fontan. We hope that the specific electrocardiographic findings presented will allow for their recognition.
Asunto(s)
Procedimiento de Fontan , Atresia Tricúspide , Adulto , Arritmias Cardíacas , Electrocardiografía , Femenino , Atrios Cardíacos/cirugía , Humanos , Atresia Tricúspide/cirugíaRESUMEN
OBJECTIVES: This study aimed to develop non-invasive machine learning classifiers for predicting post-Glenn shunt patients with low and high risks of a mean pulmonary arterial pressure (mPAP) > 15 mmHg based on preoperative cardiac computed tomography (CT). METHODS: This retrospective study included 96 patients with functional single ventricle who underwent a bidirectional Glenn procedure between November 1, 2009, and July, 31, 2017. All patients underwent post-procedure CT, followed by cardiac catheterization. Overall, 23 morphologic parameters were manually extracted from cardiac CT images for each patient. The Mann-Whitney U or chi-square test was applied to select the most significant predictors. Six machine learning algorithms including logistic regression, Naive Bayes, random forest (RF), linear discriminant analysis, support vector machine, and K-nearest neighbor were used for modeling. These algorithms were independently trained on 100 train-validation random splits with a 3:1 ratio. Their average performance was evaluated by area under the curve (AUC), accuracy, sensitivity, and specificity. RESULTS: Seven CT morphologic parameters were selected for modeling. RF obtained the best performance, with mean AUC of 0.840 (confidence interval [CI] 0.832-0.850) and 0.787 (95% CI 0.780-0.794); sensitivity of 0.815 (95% CI 0.797-0.833) and 0.778 (95% CI 0.767-0.788), specificity of 0.766 (95% CI 0.748-0.785) and 0.746 (95% CI 0.735-0.757); and accuracy of 0.782 (95% CI 0.771-0.793) and 0.756 (95% CI 0.748-0.764) in the training and validation cohorts, respectively. CONCLUSIONS: The CT-based RF model demonstrates a good performance in the prediction of mPAP, which may reduce the need for right heart catheterization in post-Glenn shunt patients with suspected mPAP > 15 mmHg. KEY POINTS: ⢠Twenty-three candidate descriptors were manually extracted from cardiac computed tomography images, and seven of them were selected for subsequent modeling. ⢠The random forest model presents the best predictive performance for pulmonary pressure among all methods. ⢠The computed tomography-based machine learning model could predict post-Glenn shunt pulmonary pressure non-invasively.
Asunto(s)
Presión Sanguínea , Procedimiento de Fontan , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Arteria Pulmonar/diagnóstico por imagen , Máquina de Vectores de Soporte , Adolescente , Algoritmos , Teorema de Bayes , Cateterismo Cardíaco , Niño , Preescolar , Análisis Discriminante , Ventrículo Derecho con Doble Salida/diagnóstico por imagen , Ventrículo Derecho con Doble Salida/cirugía , Femenino , Defectos de los Tabiques Cardíacos/diagnóstico por imagen , Defectos de los Tabiques Cardíacos/cirugía , Humanos , Lactante , Modelos Logísticos , Pulmón , Aprendizaje Automático , Masculino , Pronóstico , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugía , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodos , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/cirugía , Atresia Tricúspide/diagnóstico por imagen , Atresia Tricúspide/cirugía , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/cirugía , Adulto JovenRESUMEN
BACKGROUND: Fenestration of the baffle/conduit is believed to reduce pleural effusion following the Fontan operation. However, equivocal results have been observed with or without fenestration. This study aims to evaluate the efficacy of fenestration on the amount and duration of pleural effusion following the Fontan operation. METHODS: About 40 patients undergoing extracardiac Fontan (ECF) were randomized into two groups: one with fenestration (ECF-F; n = 20) or without fenestration (ECF-NF; n = 20). Primary outcome was the amount and duration of pleural effusions. Secondary outcomes were time to removal of the chest tubes, hospital stay, and readmission to the hospital because of recurrent pleural within 30 days of the operation. RESULTS: Mean age was 11.5 ± 5.07 (range, 8.7-13.5) years in the ECF-F group and 13.6 ± 0.4 years (range, 10.5-15.5) in the (ECF-NF) group. The total drain output was 7.89 mL/kg/d in ECF-NF compared with 6.9 mL/kg/d in the ECF-group (P = .14). Time for removal of pleural tubes was 14.6 ± 0.95 days in the ECF-NF group compared with 11.6 ± days in the ECF-F group. Total duration of hospital stay was higher but not significant in the ECF-NF group compared with the ECF-F group. Two patients in ECF-NF required readmission to the hospital within 30 days following discharge, while there were no readmissions in the ECF-F group. CONCLUSION: Contrary to the literature, the creation of a fenestration in the ECF circuit was not clearly associated with a reduction in the amount and duration of pleural effusion compared with a non-fenestrated Fontan. These findings may be debatable in high risks versus low risk candidates. However in the present study, in a low risk canditates undergoing the Fontan operation, the daily amount of pleural drainage was no different. Larger studies are needed to confirm these findings.
Asunto(s)
Procedimiento de Fontan/métodos , Derrame Pleural/prevención & control , Complicaciones Posoperatorias/prevención & control , Atresia Tricúspide/cirugía , Adolescente , Tubos Torácicos , Niño , Remoción de Dispositivos , Femenino , Humanos , Tiempo de Internación , Masculino , Estudios Prospectivos , Recurrencia , Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. This literature review considers the pathophysiology, presentation, investigations, and classification of TA. Moreover, it discusses the evidence upon which the latest medical and surgical treatments are based, as well as numerous recent case reports. Further work is needed to elucidate the etiology of TA, clarify the role of pharmacotherapy, and optimize the surgical management that these patients receive.
Asunto(s)
Procedimiento de Fontan/métodos , Atresia Tricúspide/cirugía , Válvula Tricúspide/cirugía , Alprostadil/uso terapéutico , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Preescolar , Femenino , Procedimiento de Fontan/mortalidad , Humanos , Lactante , Recién Nacido , Masculino , Cuidados Posoperatorios , Tasa de Supervivencia , Resultado del Tratamiento , Atresia Tricúspide/clasificación , Atresia Tricúspide/diagnóstico , Atresia Tricúspide/mortalidadRESUMEN
Tricuspid valve atresia with severe pulmonary stenosis is one of the common cyanotic diseases in neonate. Child can succumb due to profound cyanosis and arterial hypoxaemia after closure of patent ductus arteriosus. Evolving procedure of right ventricular outflow tract stenting may be considered as a palliative procedure in such vulnerable group, destined for a later definitive management. The right ventricular outflow tract stenting is described essentially for tetralogy of Fallot physiology with a catheter course across tricuspid valve. We describe a case of successful right ventricular outflow tract stenting in a 5-day-old symptomatic neonate. We discuss the possible routes and the tips to facilitate right ventricular outflow tract stenting in such a case. This happens to be the first reported case description with successful stenting of neonate with tricuspid atresia with critical pulmonic stenosis.
Asunto(s)
Conducto Arterioso Permeable , Atresia Pulmonar , Estenosis de la Válvula Pulmonar , Tetralogía de Fallot , Atresia Tricúspide , Niño , Cianosis , Ventrículos Cardíacos , Humanos , Recién Nacido , Estenosis de la Válvula Pulmonar/complicaciones , Estenosis de la Válvula Pulmonar/diagnóstico , Estenosis de la Válvula Pulmonar/cirugía , Atresia Tricúspide/complicaciones , Atresia Tricúspide/cirugíaRESUMEN
Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.
Asunto(s)
Procedimiento de Fontan/métodos , Válvula Pulmonar/anomalías , Atresia Tricúspide/complicaciones , Tabique Interventricular , Puente Cardiopulmonar , Ecocardiografía Doppler , Ecocardiografía Tridimensional , Humanos , Hipertrofia Ventricular Derecha/diagnóstico , Hipertrofia Ventricular Derecha/etiología , Lactante , Masculino , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Atresia Tricúspide/diagnóstico , Atresia Tricúspide/cirugíaRESUMEN
BACKGROUND: Total Cavopulmonary connection (Fontan) is the final palliation for patients with a functionally univentricular heart. This is commonly accomplished after a prior bidirectional Glenn on cardiopulmonary bypass (CPB) with separate cannulation of the aorta, superior vena cava (SVC), and inferior vena cava. We describe an alternative technique of Fontan completion that eliminates the need for cannulation and dissection of the SVC, and pulmonary artery dissection. METHODS: Between January and October 2018, 17 patients underwent completion Fontan using an alternate technique at our institute. All operations were conducted on CPB at normothermia without cannulating the SVC RESULTS: Mean CPB time was 60 ± 16.8 minutes (range, 39-102 minutes). There were no early deaths. Mean postoperative Fontan pressures were 15.6 ± 1.2 mm Hg with no gradient between the SVC and IVC pressures. Mean duration of hospital stay was 15.6 ± 3.6 days (range, 10-22 days). No patient developed phrenic nerve paresis or palsy. CONCLUSIONS: Completion without cannulating the SVC is simple, reproducible, and easy to teach. It avoids the disadvantages associated with routine techniques.
Asunto(s)
Procedimiento de Fontan/métodos , Adolescente , Puente Cardiopulmonar/métodos , Cateterismo/métodos , Niño , Ventrículo Derecho con Doble Salida/cirugía , Femenino , Defectos del Tabique Interventricular/cirugía , Humanos , Masculino , Estenosis de la Válvula Pulmonar/cirugía , Transposición de los Grandes Vasos/cirugía , Resultado del Tratamiento , Atresia Tricúspide/cirugía , Vena Cava Superior , Adulto JovenRESUMEN
BACKGROUND: Increased pulmonary vascular resistance index (PVR) leads to several complications in patients after a Fontan operation. This increase is mainly attributed to the overexpression of endothelin-1 for a long duration after the Fontan procedure. Here, we describe the case of a 3-year-old boy with a failed Fontan operation who was treated with bosentan, an endothelin-1 receptor blocker. CASE REPORT: Cardiac catheterization was performed, which showed a main pulmonary artery pressure (MPAP) of 19 mmHg and PVRI of 5.6 woods/m2. Oral bosentan regimen at a dose of 31.25 mg was initiated twice a day. The treatment was continued as pleural effusion and ascites persisted. No adverse events were observed, and the treatment was well tolerated. Pleural effusion disappeared, and ascites decreased markedly after 4 weeks, whereas the MPAP was 15 mmHg and the PVRI was 4.3 woods/m2. After 3 months of bosentan therapy, the MPAP was 12 mmHg and the PVRI was 4.1 woods/m2. CONCLUSION: We observed that bosentan reduces the PVRI and complications such as pleural effusion and ascites after a failed Fontan procedure.
Asunto(s)
Bosentán/uso terapéutico , Antagonistas de los Receptores de Endotelina/uso terapéutico , Procedimiento de Fontan/efectos adversos , Complicaciones Posoperatorias/tratamiento farmacológico , Atresia Tricúspide/cirugía , Resistencia Vascular , Preescolar , Humanos , Masculino , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/fisiopatología , Insuficiencia del Tratamiento , Atresia Tricúspide/complicaciones , Atresia Tricúspide/fisiopatologíaRESUMEN
A 42-year-old man was referred to our hospital with heart failure and unoperated tricuspid atresia with pulmonary valve stenosis. His condition was initially managed with medical therapy; however, he required repeat hospitalisations for congestive heart failure. We diagnosed the chief cause of his heart failure as aortic valve regurgitation secondary to aortic root dilatation. Aortic root replacement was performed and then his heart failure was controlled.
Asunto(s)
Aorta Torácica/cirugía , Prótesis Vascular , Procedimientos Quirúrgicos Cardíacos/métodos , Atresia Tricúspide/cirugía , Adulto , Aorta Torácica/diagnóstico por imagen , Ecocardiografía , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/cirugía , Humanos , Masculino , Tomografía Computarizada por Rayos X , Atresia Tricúspide/complicaciones , Atresia Tricúspide/diagnósticoRESUMEN
BACKGROUND: The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (VÌo2). METHODS: We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between VÌo2 and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak VÌo2 and LV function indices. RESULTS: Seventy-five patients (44 men; 59%) underwent 191 pairs of TTE and CPET. At baseline, mean age was 24±3 years, peak VÌo2 was 22.9±4.1 mL/kg/min (63±11 percent predicted), SVI was 43±15 mL/m2, and CI was 2.9±0.9 L/min/m2. Peak VÌo2 correlated with SVI (r=0.30, P<.001) and with CI (r=0.45, P<.001) in the 153 pairs of TTE and CPET in patients without cirrhosis. Temporal changes in percent predicted peak VÌo2 correlated with changes in SVI (r=0.48, P=.005) and CI (r=0.49, P=.004) among the 33 patients without interventions during the study. In the 19 patients with Fontan conversion, percent predicted peak VÌo2 and chronotropic index improved. CONCLUSIONS: Overall, there was a temporal decline in peak VÌo2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak VÌo2 that correlated with improvement in chronotropic index.