A Case of Isolated Bicuspid Pulmonic Valve and Pulmonary Artery Aneurysm

A 33-year-old woman with a history of thyroid surgery for thyroid cancer and radioactive iodine therapy was referred for echocardiography due to dyspnea on exertion. Transthoracic echocardiography showed normal left ventricular size and function (the ejection fraction = 55%), a prolapsing mitral valve with redundant chordae, mild mitral regurgitation, a tricuspid aortic valve, mild aortic insufficiency, and mild tricuspid regurgitation. The most remarkable echocardiographic findings were moderate right ventricular dilation with mild systolic dysfunction, moderate right atrial dilation, an aneurysmal pulmonary artery (the main pulmonary artery = 47 mm), mild pulmonary stenosis (the peak gradient = 22 mmHg), and severe pulmonary regurgitation (the vena contracta = 6–7 mm and the pressure half time = 105 ms). Transesophageal echocardiography with the use of 3D modalities demonstrated a bicuspid pulmonic valve with doming and poor coaptation of the pulmonic valve leaflets (Figure 1). Additionally, a large patent foramen ovale was visualized in color Doppler (the flap separation = 2 mm and the tunnel length = 11 mm) with bubble passage in agitated saline injection. Bicuspid pulmonic valves constitute a rare finding, and they are most often associated with other congenital heart diseases. Isolated bicuspid pulmonic valves are extremely rare, with an incidence rate of about 0.1% in clinical practice.1 Pulmonary artery aneurysms also comprise a rare abnormality, with an incidence rate of approximately 1 in 14 000 cases in most studies.2 The association between bicuspid pulmonic valves and pulmonary artery aneurysms has been reported, and the pathophysiologic causes of this association include hemodynamic alterations due to bicuspid pulmonic valves and most likely the abnormal migration of neural crest cells.3 The diagnosis of a bicuspid pulmonic valve by 2D imaging is challenging and sometimes impossible. Using 3D echocardiography and reconstruction confers a better assessment of the pulmonic valve morphology and identification of bicuspid pulmonic valves.

672 Isolated pulmonic valve endocarditis. AN unusual case with giant vegetations detected by transthoracic echocardiography

Aims Isolated pulmonic valve infective endocarditis (PV-IE) is a rare form of endocarditis. Methods and results The authors report a case of giant vegetations detected by transthoracic echocardiography on pulmonic valve in a 33 year-old patient, with drug abuse history (Figure 1). Patient underwent surgical intervention by pulmonary valved bioconduit implantation. After operation a pulmonary embolism episode was treated by a direct oral anticoagulant. The final outcome was favourable. We report a short review of this rare pathology. Conclusions The difficulty in diagnosing PV-IE is due to inability to properly visualize the pulmonic valve by echocardiography. In this case, with such large vegetations, transthoracic echocardiography (TTE) allowed a correct diagnosis and an effective surgical planning, confirming its importance as diagnostic tool.

Multicentre comparative analysis of long-term outcomes after aortic valve replacement in children

ObjectiveThe ideal valve substitute for surgical intervention of congenital aortic valve disease in children remains unclear. Data on outcomes beyond 10–15 years after valve replacement are limited but important for evaluating substitute longevity. We aimed to describe up to 25-year death/cardiac transplant by type of valve substitute and assess the potential impact of treatment centre. Our hypothesis was that patients with pulmonic valve autograft would have better survival than mechanical prosthetic.MethodsThis is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional US-based registry of paediatric cardiac interventions, linked with the National Death Index and United Network for Organ Sharing through 2019. Children (0–20 years old) receiving aortic valve replacement (AVR) from 1982 to 2003 were identified. Kaplan-Meier transplant-free survival was calculated, and Cox proportional hazard models estimated hazard ratios for mechanical AVR (M-AVR) versus pulmonic valve autograft.ResultsAmong 911 children, the median age at AVR was 13.4 years (IQR=8.4–16.5) and 73% were male. There were 10 cardiac transplants and 153 deaths, 5 after transplant. The 25-year transplant-free survival post AVR was 87.1% for autograft vs 76.2% for M-AVR and 72.0% for tissue (bioprosthetic or homograft). After adjustment, M-AVR remained related to increased mortality/transplant versus autograft (HR=1.9, 95% CI=1.1 to 3.4). Surprisingly, survival for patients with M-AVR, but not autograft, was lower for those treated in centres with higher in-hospital mortality.ConclusionPulmonic valve autograft provides the best long-term outcomes for children with aortic valve disease, but AVR results may depend on a centre’s experience or patient selection.

Pulmonary Artery Aneurysm Repair with Alpha-1 Anti-trypsin Deficiency

A 62-year-old woman with pulmonary hypertension due to alpha-1 antitrypsin deficiency and known congenital pulmonic valve stenosis presented with palpitations, chest pressure and bradycardia and was found to have a 6 cm pulmonary artery aneurysm on work-up. The patient underwent surgical pulmonary artery aneurysm repair and recovered from operation without complications.

Risk factors and long-term outcomes after tetralogy of Fallot repair at an Asian tertiary referral center

Background Tetralogy of Fallot is the most common type of cyanotic congenital heart disease. More postoperative tetralogy of Fallot patients grow up than in the past, and these patients need to be followed-up. Objective To investigate the survival and long-term outcomes of patients who underwent total repair of tetralogy of Fallot, and to identify the risk factors for reoperation with pulmonic valve replacement. Method A total of 403 patients who underwent total tetralogy of Fallot repair at our center during 1997 to 2016 were retrospectively included. Demographic, clinical, treatment, outcome, and follow-up data were collected and analyzed. Results Median age and body weight at the time of tetralogy of Fallot repair was 4.41 years (range: 0.85–55.28) and 13.58 kg (range: 5.5–68), respectively. The median follow-up was 9.0 years, and overall mortality was 3.2%. The actuarial survival rates at 10 and 20 years were 96.4% and 95.2%, respectively, and the freedom from pulmonic valve replacement was 93.4% and 57.4%, respectively. The median time to indicate pulmonic valve replacement was 13.9 years (range: 6.2–20.5). Multivariate analysis revealed transannular patch technique (hazard ratio: 3.023, 95% confidence interval: 1.34–6.83; p = 0.008) and palliative shunt (hazard ratio: 2.39, 95% confidence interval: 1.16–4.91; p = 0.018) to be independent risk factors for reoperation with pulmonic valve replacement. Conclusion The rates of overall survival and freedom from pulmonic valve replacement were both high in this study, and both were comparable to the rates reported from other studies. Overall mortality was as low as 3.47%. The need for a transannular patch or palliative shunt should be considered risk factors for a consequent reoperation.