Project description:We report the case of a successful complex percutaneous intervention in a patient with Fontan circulation and severe heart failure. The patient presented with cyanosis; Fontan conduit stenosis was detected, and the fenestration was patent. The complex interventional procedure allowed for a long-term stabilization of the patient's condition.
Project description:BackgroundThe frequency of arrhythmias increases after the Fontan operation over time; atrial tachycardia (AT) and sinus node dysfunction (SND) are frequently observed.Case summaryOur patient was 63-year-old woman who underwent a lateral tunnel Fontan operation for double outlet right ventricle at age 36. She experienced paroxysmal AT for 1 year, and antiarrhythmic medication was not feasible due to symptomatic SND. Computed tomography revealed a 45 mm-sized thrombus in the high right atrium (RA). The patient had three coexisting conditions: paroxysmal AT, symptomatic SND, and the right atrial thrombus, for which total cavopulmonary connection conversion and epicardial pacemaker implantation (PMI) would have been effective; however, given her age and comorbidities, surgical treatment was considered high risk. Catheter ablation was avoided because of the right atrial thrombus. Finally, a transvenous pacemaker was implanted via the right femoral vein to avoid the right atrial thrombus and severe venous tortuosity from the left subclavian vein to the RA. After PMI, the patient was prescribed amiodarone and bisoprolol for AT suppression. Atrial tachycardia occurred once in the third month after discharge. We increased the dose of amiodarone, and she has been tachycardia-free.DiscussionTransvenous PMI must be considered in cases where open thoracic surgery or catheter ablation cannot be performed. This is the first report of transvenous PMI via the right femoral vein and successful AT and SND management in an elderly Fontan patient.
Project description:BackgroundThere is limited evidence for the use of an intra-aortic balloon pump (IABP) in adult patients with a total cavopulmonary, or Fontan circulation.Case summaryA patient in his twenties with a Fontan circulation presented with sepsis, pneumonia, and pulmonary oedema. He was born with a hypoplastic left ventricle, atrioventricular septal defect, and hypoplastic aortic arch, and a total cavopulmonary circulation had been established within his first years of life. Standard of care treatment with antibiotics, non-invasive ventilatory support, loop diuretics, and vasopressors was initiated. Due to persistent pulmonary congestion and increasing general fatigue, an IABP was placed via a femoral artery to offload the failing systemic ventricle. Secondary to IABP treatment, mean arterial pressure rose, and vasodilatory nitroprusside could be introduced. Over 4 days of IABP treatment, the patient's general condition and ventricular systolic function improved significantly.DiscussionThis case suggests that IABP treatment was important in the recovery of our patient with a Fontan circulation, pneumonia, and heart failure. We propose that during IABP treatment, an increase in stroke volume and a reduction in ventricular filling pressure is achieved, thereby increasing the transpulmonary pressure gradient that is central to pulmonary blood flow in Fontan patients. More definitive evidence is necessary to confirm our hypotheses.
Project description:BackgroundAt the most severe end of the spectrum of congenital heart disease are patients with an univentricular physiology. They comprise a heterogeneous group of congenital heart malformations that have the common characteristic that the cardiac morphology is not equipped for sustaining a biventricular circulation.Case summaryHere, we present a case of an adult patient after Fontan palliation, illustrative of the complex clinical course and the broad spectrum of complications that can be encountered during follow-up, highlighting the need for a multidisciplinary approach in the clinical care for these patients.DiscussionDuring the surgical Fontan procedure, the inferior vena cava is connected to the pulmonary circulation, after prior connection of the superior vena cava to the pulmonary arterial circulation. The resulting cavopulmonary connection, thus lacking a subpulmonic ventricle, provides non-pulsatile passive flow of oxygen-poor blood from the systemic venous circulation into the lungs, and the functional monoventricle pumps the oxygen-rich pulmonary venous return blood into the aorta. With an operative mortality of <5% and current 30-year survival rates up to 85%, the adult population of patients with a Fontan circulation is growing. This increase in survival is, however, inevitably accompanied by long-term complications affecting multiple organ systems, resulting in decline in cardiovascular performance.ConclusionFor optimal treatment, the evaluation in a multidisciplinary team is mandatory, using the specific expertise of the team members to timely detect and address late complications and to support quality of life.
Project description:BackgroundFontan circulation is created when a baby is born with only one functioning cardiac ventricle. A series of surgeries are performed to allow the ventricle to provide oxygenated blood to the systemic circulation and to create passive flow of venous blood to the pulmonary circulation via a conduit. Laparoscopic surgery poses several hemodynamic challenges to a patient with Fontan physiology attributable to carbon dioxide insufflation, positive pressure ventilation, and reverse Trendelenburg positioning.Clinical featuresA 39-yr-old male with a Fontan physiology was referred to our tertiary care centre because of repeated bouts of cholecystitis requiring a percutaneous drain and now elective laparoscopic cholecystectomy. Because of repeated cardiac surgeries, the patient also had complete heart block and was pacemaker dependent. We placed an arterial catheter prior to induction of general anesthesia with tracheal intubation. Transesophageal echocardiography allowed for real-time intraoperative assessment of venous blood flow through the patient's extracardiac diversion system throughout the surgery. This information was used to guide management and determine circulation tolerance during the various stages of laparoscopy. Inhaled milrinone resulted in the shunt fraction returning to the patient's baseline. Intraperitoneal pressure was kept below 10 mm Hg, and systemic blood pressure was supported with a low-dose norepinephrine infusion.ConclusionsIntraoperative transesophageal echocardiography is a useful monitoring device during laparoscopic surgery when a patient has Fontan circulation. Knowing how to administer inhaled milrinone is a useful skill to decrease the shunt fraction through a patient's conduit, increasing pulmonary blood flow while avoiding hypotension.
Project description:Fontan physiology creates a chronic state of decreased cardiac output and systemic venous congestion, leading to liver cirrhosis/malignancy, protein-losing enteropathy, chylothorax, or plastic bronchitis. Creating a fenestration improves cardiac output and relieves some venous congestion. The anatomic connection of the thoracic duct to the subclavian-jugular vein junction exposes the lymphatic system to systemic venous hypertension and could induce plastic bronchitis. To address this complication, two techniques have been developed. A surgical method that decompresses the thoracic duct by diverting the innominate vein to the atrium, and a percutaneous endovascular procedure that uses a covered stent to create an extravascular connection between the innominate vein and the left atrium. We report a novel variant transcatheter intervention of the innominate vein turn-down procedure without creating an extravascular connection in a 39-month-old patient with failing Fontan circulation complicated by plastic bronchitis and a 2-year post-intervention follow-up.
Project description:Few studies have investigated how the Fontan circulation affects lung function, and no studies have investigated the development of lung function over longer time in these patients. We aimed to describe the development of lung function in Fontan patients over a 10-year period. Pulmonary function tests (PFT), including spirometry and diffusion capacity for Carbon Monoxide (DLCO) and Nitric Oxide (DLNO), were conducted in a Danish Fontan cohort in 2011 (PFT-I). In 2021, re-investigations were performed (PFT-II). We investigated changes in percent predicted (%pred) lung function from PFT-I to PFT-II. Patients were categorized into a pediatric group (age under 18 at PFT-I) and an adult group (age 18 or older at PFT-I). Out of the 81 patients completing PFT-I, 48 completed PFT-II. In the pediatric group (32 patients), there were significant declines in %pred forced expiratory volume in 1s (99.7 (92.4, 104.4)-89.3 (84.9, 97.2), p < 0,001), forced vital capacity (98.3 (87.8, 106.1)-96.7 (86.7, 100.6), p = 0.008), and alveolar volume (95.5 (89.5, 101.6)-89.5 (79.7, 93.2), p < 0.001). The corresponding measurements remained stable in the adult group. However, the median %pred DLNO significantly declined in the adult group (58.4 (53.3, 63.5)-53.7 (44.1, 57.3), p = 0.005). Over a 10-year period, several lung function parameters declined significantly in the younger Fontan patients, suggesting possible impairments in lung development during growth. The decline in %pred DLNO in the adult patient group indicates deterioration of the membrane component of diffusion capacity, implying that the Fontan circulation might negatively affect the alveolar membrane over time.
Project description:BackgroundTracheobronchomegaly (TBM) is a rare disorder mainly characterized by dilatation and malacia of the trachea and major bronchi with diverticularization. This will be a great challenge for airway management, especially in thoracic surgery requiring one-lung ventilation. Using a laryngeal mask airway and a modified double-lumen Foley catheter (DFC) as a "blocker" may achieve one-lung ventilation. This is the first report introducing this method in a patient with TBM.Case presentationWe present a 64-year-old man with TBM receiving left lower lobectomy. Preoperative chest computed tomography demonstrated a prominent tracheobronchial dilation and deformation with multiple diverticularization. The most commonly used double-lumen tube or bronchial blocker could not match the distorted airways. After general anesthesia induction, a 4# laryngeal mask was inserted, through which the modified DFC was positioned in the left main bronchus with the guidance of a fiberoptic bronchoscope. The DFC balloon was inflated with 10 ml air and lung isolation was achieved without any significant air leak during one-lung or two-lung ventilation. However, the collapse of the non-dependent lung was delayed and finally achieved by low-pressure artificial pneumothorax. The surgery was successful and the patient was extubated soon after the surgery.ConclusionsUsing a laryngeal mask airway with a modified double-lumen Foley catheter acted as a bronchial blocker could be an alternative method to achieve lung isolation.
Project description:BackgroundThe use of transcatheter edge-to-edge repair (TEER) in patients with advanced heart failure has been shown to reduce hospitalizations and increase survival. As patients with Fontan circulations grow older, a significant proportion of them will develop severe atrioventricular (AV) valve regurgitation in the systemic ventricle. Conventional surgical repair and transplant carry high mortality risk for the adult Fontan patient with progressive heart failure.Case summaryA 51-year-old female extracardiac Fontan patient developed severe AV valve regurgitation and progressive functional decline. Based on her operative risk for conventional surgical intervention or transplant, TEER using the Abbott MitraClip device was performed. The degree of mitral regurgitation was decreased from severe to moderate regurgitation.DiscussionThis is the first known case describing the use of a successful TEER in an adult patient with an extracardiac Fontan. Given the increasing numbers of patients surviving into adulthood with a Fontan circulation, transcatheter interventions may provide an alternative treatment option to conventional surgeries and medical therapies.