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Paediatrics
Published in Vincent Helyar, Aidan Shaw, The Final FRCR, 2017
This is the second most common cause of neonatal cyanosis after transposition. It is characterised by a completely absent tricuspid valve, an ASD and a small VSD. Most occur in the absence of transposition (80%). Treatment with the Fontan procedure is at 3–5 years of age. The pulmonary artery is anastomosed to the right atrium.
Interventional cardiac catheterisation in adults with congenital heart disease
Published in Ever D. Grech, Practical Interventional Cardiology, 2017
Hussam S Suradi, Ziyad M Hijazi
The growing number of patients who have palliated single ventricle physiology is a group with significant adult onset complications. These patients typically have undergone a variation of the Fontan procedure to shunt all the systemic venous blood directly into the pulmonary arteries. Common indications for catheterisation in the adults with Fontan palliation are for haemodynamic evaluation in case of clinical status change like dysrhythmia, and for preoperative assessment prior to Fontan revision or cardiac transplant. During catheterisation, it is important to record pulmonary, Fontan and aortic pressures as well as to evaluate for the presence of shunting (right-to-left or left-to-right) with oxygen saturation assessment at various levels. Angiograms should be performed to define the anatomical details of the great vessels, collateral vessels, the systemic venous drainage as well as the left ventricular volume and ejection fraction. The low, non-pulsatile flow in the systemic venous circulation and multiple suture lines can lead to significant stenosis within the Fontan circulation (see Figure 36.13). The same techniques of balloon angioplasty and stent placement are applied to relieve any anatomical obstruction in this complicated patient population. Similarly, trans-catheter closure using coils and occlusion devices are applied for abnormal vascular connections closure that may cause right-to-left (causing cyanosis and paradoxical embolism) or left-to-right shunting (causing systemic ventricular volume overload) (see Figures 36.14 and 36.15).
Cardiac catheterization for the adult with complex congenital heart disease
Published in Debabrata Mukherjee, Eric R. Bates, Marco Roffi, Richard A. Lange, David J. Moliterno, Nadia M. Whitehead, Cardiovascular Catheterization and Intervention, 2017
A patient with a functional single ventricle typically proceeds with palliation, including the systemic shunt (Norwood opera- tion, Potts shunt, Waterston shunt, or other central shunts), followed by the SVC shunt or Glenn operation, and ultimately, the completion of the caval pulmonary shunt with the Fontan operation (Figure 20.10). Congenital defects that may neces- sitate such palliation include hypoplastic left heart, tricuspid atresia, pulmonary atresia with intact ventricular septum, or unbalanced complete AV canal. The ACHD patient with single-ventricle physiology is usually palliated with a Fontan, but few patients remain with a single lung supplied by a SVC to the pulmonary artery shunt (Glenn) or a systemic artery to pulmonary artery shunt. The Fontan procedure represents the final palliative procedure for single-ventricle physiologic status. This procedure completes the direction of the remain- ing systemic venous blood from the IVC and hepatic veins to the pulmonary arteries. This is accomplished in most cases by either an external conduit or an intraatrial lateral tunnel, which courses from the lateral and inferior aspect of the RA. [30]The atrial appendage or superior vena caval stump transected during the Glenn procedure is directed to the pulmonary artery, effectively “septating” the circulation. Pulmonary blood flow is achieved passively without the assistance of a ventricu- lar pumping chamber. For this reason, it is imperative to have low pulmonary pressures and vascular resistance.
The evolution of long-term pediatric ventricular assistance devices: a critical review
Published in Expert Review of Medical Devices, 2021
Louis Marcel, Mathieu Specklin, Smaine Kouidri
The various surgical interventions in young patients result in a modified anatomy of the heart, of the systemic and pulmonary circulation. For instance, the Fontan procedure is a surgical intervention used in children suffering from various CHDs such as triscupid atresias (absence of right atrioventricular connection) or univentricular hearts. The interventions modifies the blood circulation around and inside the heart and is described in Figure 4. The venous blood coming from superior and inferior vena cava is diverted directly to the pulmonary arteries without passing through the ventricle. Pulmonary and systemic circulation are then placed in series with the dingle ventricle. As devices are designed for normal morphology, patients that have completed the Fontan operation present an inferior surival compared to those with bi-ventricular physiology (42% vs 73%) [47]. [48; 49; 50; 51; 52; 53; 54]
Anesthetic management of a single ventricle pediatric patient with a major burn injury, case report
Published in Egyptian Journal of Anaesthesia, 2020
Ahmed Haroun M. Mahmoud, Ahmad Tareq Aboalfaraj, Turki Abdullah Almalki, Ahmed Mounir A. Metwally
The Glenn procedure is one of the steps of palliative surgeries performed to correct hypoplastic left heart syndrome and other single- ventricle heart defects. It is usually performed about six months after birth. In this operation, the superior vena cava is connected to the right pulmonary artery. Venous return from the head and upper limbs passively flow into the pulmonary artery and proceeds to the lungs to be oxygenated. However, oxygen-poor blood returning to the heart from the lower body through the inferior vena cava will mix with oxygen-rich blood in the left heart and circulate to the body so that the saturation remains in the low 80% range. This operation is preparation for the final step which is the Fontan procedure [1–3].
Rivaroxaban for the treatment of venous thromboembolism in pediatric patients
Published in Expert Review of Cardiovascular Therapy, 2020
Omri Cohen, Sarina Levy-Mendelovich, Walter Ageno
Single Ventricle physiology represents 10% of congenital heart disease. Fontan procedure is the final step of a 3-stage palliative procedure commonly performed in children with single ventricle physiology; however, thrombosis is a potential complication [52]. Type and duration of anticoagulant treatment are extrapolated from other clinical settings. Current guidelines recommend the use of either aspirin or UFH followed by VKAs for thromboprophylaxis in children undergoing the Fontan procedure, nonetheless, these are primarily expert opinions based on limited literature [1].