A rare cause of Right ventricular outflow tract Pseudoaneurysm in Tetralogy of Fallot

Pseudoaneurysm of the right ventricular outflow tract(RVOT) is an uncommon yet catastrophic complication after intracardiac repair of Tetralogy of Fallot(TOF). We describe a patient diagnosed with RVOT pseudoaneurysm in the immediate postoperative period after complete repair for TOF with single pulmonary artery. The pseudoaneurysm was repaired successfully. This case is reported to emphasise the importance of a high degree of suspicion of this rare entity in these patients for its early diagnosis and management.

‘Superimposed miracles’ An extremely rare case of a septuagenarian with an unrepaired tetralogy of Fallot: a case report

Background Although the life expectancy of patients with tetralogy of Fallot (TOF) is comparable to that of the general population due to advancements in surgical intervention, if untreated, patients with TOF may die during their childhood. However, it has been anecdotally reported that a small number of patients with unrepaired TOF survived into their senescence. Case Summary A 71-year-old man with a history of multiple heart failure admissions was referred to our institute after successful cardiopulmonary arrest resuscitation. Transthoracic echocardiography showed the overriding of the aorta on a large ventricular septal defect and right ventricular hypertrophy, along with severe pulmonary stenosis (PS), all of which indicated unrepaired TOF. Computed tomography revealed a patent Blalock-Taussig shunt, which was constructed at the age of 19 years. Coronary angiography revealed multivessel coronary stenoses. Although radical intracardiac repair was not performed due to his multiple comorbidities, his heart failure symptoms were significantly improved owing to proper medication titration. One year following discharge, the patient was well and enjoyed playing golf. Discussion Specific anatomical, functional and haemodynamic characteristics may be required for the long-term survival of patients with TOF. PS should be initially mild to guarantee pulmonary flow during childhood and adolescence, and gradual PS exacerbation should be paralleled with systemic-to-pulmonary collateral development or an extra-cardiac shunt. Moreover, reduced left ventricular compliance may act as a balancing factor against a right-to-left shunt. The presence of all of these special requirements may have contributed to the unusual survival for this patient.

Incidence of Infective Endocarditis Among Patients With Tetralogy of Fallot

Background Patients with tetralogy of Fallot (ToF) are considered at high risk of infective endocarditis (IE) as a result of altered hemodynamics and multiple invasive procedures, including pulmonary valve replacement (PVR). Data on the long‐term risk of IE are sparse. Methods and Results In this observational cohort study, all patients with ToF born from 1977 to 2018 were identified using Danish nationwide registries and followed from date of birth until occurrence of first‐time IE, emigration, death, or end of study (December 31, 2018). The comparative risk of IE among patients with ToF versus age‐ and sex‐matched controls from the background population was assessed. Because of rules on anonymity, exact numbers cannot be reported if the number of patients is <4. A total of 1164 patients with ToF were identified and matched with 4656 controls. Among patients with ToF, 851 (73.1%) underwent early surgical intracardiac repair and 276 (23.7%) underwent PVR during follow‐up. During a median follow‐up of 20.3 years, 41 (3.5%) patients with ToF (comprising 24 [8.7%] with PVR and 17 [1.9%] without PVR) and <4 (<0.8%) controls were admitted with IE. The incidence rates of IE per 10 000 person‐years were 22.4 (95% CI, 16.5–30.4) and 0.1 (95% CI, 0.01–0.7) among patients and controls, respectively. Moreover, PVR was associated with a further increased incidence of IE among patients with ToF (incidence rates per 10 000 person‐years with and without PVR were 46.7 [95% CI, 25.1–86.6] and 2.8 [95% CI 2.0–4.0], respectively). Conclusions Patients with ToF are associated with a substantially higher incidence of IE than the background population. In particular, PVR was associated with an increased incidence of IE. With an increasing life expectancy of these patients, intensified awareness, preventive measures, and surveillance of this patient group are decisive.

Left Pulmonary Artery Sling: Postoperative Outcomes for Patients at a Single Center

Background Left pulmonary artery (LPA) sling is a rare anomaly characterized by the origin of the LPA from the right pulmonary artery with a course between the trachea and esophagus. It is often associated with airway and cardiac anomalies. Methods This is a retrospective case series of consecutive patients who underwent LPA sling repair (LPASR) at a tertiary care center over a 35-year period with a focus on tracheal and/or LPA reinterventions and survival. Results Between June 1983 and July 2018, 42 patients were identified: isolated LPASR was performed in 16 (38%), LPASR/intracardiac repair in 10 (24%), and LPASR/tracheal repair in 16 (38%). There were 5 (12%) in-hospital and 4 (10%) late deaths. Survival rates (15 years) were as follows: 100% (isolated LPASR), 65% (concomitant intracardiac repair), and 52% (concomitant tracheal surgery). Preoperative intensive care unit (ICU) hospitalization was associated with future intervention on the LPA/trachea (61%, 11/18). The median distribution of blood flow to the left lung post-index surgery was 38%. Freedom from isolated LPA intervention was 100% after isolated LPASR, 93% after LPASR/tracheal surgery, and 69% after LPASR/intracardiac repair. Freedom from isolated tracheal intervention was 92% after isolated LPASR, 73% after LPASR/tracheal surgery, and 78% after LPASR/intracardiac repair. Conclusions ICU hospitalization prior to index surgery may indicate the severity of associated cardiac/tracheal abnormalities as this is associated with increased morbidity and mortality. Patients who underwent LPASR/intracardiac surgery were more likely to undergo isolated LPA intervention and those who underwent LPASR/tracheal surgery were more likely to undergo isolated tracheal intervention.

Perioperative Anesthetic Management for Surgical Repair of an Adult with Supracardiac Total Anomalous Pulmonary Venous Communication and Pulmonary Hypertension: A case report and Mini Review

The total anomalous pulmonary venous communication [TAPVC] is a rare cyanotic congenital cardiac defect accounting for 1.5-3% of the congenital heart disease, in which pulmonary venous [PV] blood drains directly into the right side of the heart or into the systemic veins. Neonates with obstructive TAPVC may present with cyanosis, metabolic acidosis, respiratory failure, and shock. A subset of patients with unobstructed TAPVC may remain symptoms free and attain adulthood, or present with pulmonary congestion, pulmonary arterial hypertension [PAH]. The anesthetic management of either obstructed TAPVC or unobstructed with PAH can be quite challenging. The described patient is a 23-year male who presented with self – limiting single episode of chest pain, palpitations and dyspnea, diagnosed as supracardiac unobstructed TAPVC with ostium secundum atrial septal defect [OS – ASD] and PAH, who underwent successful intracardiac repair under cardiopulmonary bypass [CPB]. The protocol for the cardiac surgery during the COVID-19 pandemic for perioperative considerations and triage recommendations was strictly followed to reduce the risk of exposure to patients and healthcare workers. The objective of this case report and review is to recognize the spectrum of various clinical presentations in TAPVC, and to describe the diagnosis and perioperative management of TAPVC. Key Words: Adult Supra cardiac TAPVC, unobstructed, PAH, cardiopulmonary bypass, corrective surgery, balanced general anesthesia

Isolated left subclavian with a compensatory ductus in a child with Tetralogy of Fallot

Tetralogy of Fallot is a cyanotic heart disease wherein aortopulmonary collaterals serve as source of pulmonary blood flow to maintain oxygenation. We report an incidentally detected isolated left subclavian artery supplying a compensatory ductus in a child with Tetralogy of Fallot that effectively contributed as a de novo palliative systemic to pulmonary artery shunt. Clinically, the entity could not be suspected, as the child did not have symptoms suggestive of arterial insufficiency of the left arm or weak pulses or neurological symptoms. The child underwent successful intracardiac repair with a reimplantation of left subclavian artery to left common carotid artery.

Repair of a Unique Sinus of Valsalva Defect in an Infant

Sinus of Valsalva aneurysm is a rare disease characterized by the partial elevation of the aortic root. Few reports are available on the surgical treatment for infants. We report the repair of an extremely rare case of a sinus of Valsalva defect with a ventricular septal defect and right ventricular outflow tract stenosis in an infant. It was not a sinus of Valsalva aneurysm, but it exhibited abnormal partial bulging of the aortic root and forming an aneurysm-like cavity within the right ventricular myocardium. We performed direct closure of the sinus of Valsalva aneurysm-like cavities and intracardiac repair in two stages. Three years after total repair, the patient remained healthy and asymptomatic.