Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management.

All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study.

We included 221 patients (116 males) with a median age of 19 (18–25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years.

Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.

The purpose of this study is to assess the feasibility, effectivity, and safety of a novel self-expandable valve system, Pulsta® transcatheter heart valve in patients with tetralogy of fallot and severe pulmonary regurgitation after transannular patch repair.

Severe pulmonary regurgitation after tetralogy of fallot repair is a life-threatening problem and should be treated by pulmonary valve implantation. Although percutaneous pulmonary valve implantation has been ever increasingly used for this purpose, available balloon-expandable valves have limitations and cannot be used by most patients. Pulsta® transcatheter heart valve is a new self-expandable valve system and offers a new solution to be used in patients with different types of native right ventricular outflow tract geometry.

Ten patients with severe regurgitation after tetralogy of fallot repair with a transannular patch have been enrolled in the study according to echocardiographic examination. MRI was used in asymptomatic patients to delineate the indication and the right ventricular outflow tract geometry. Pulsta® transcatheter heart valve implantation was performed in ten patients, and preprocedural, procedure, and 6 months follow-up findings of the patients were evaluated.

Pulsta® pulmonary valve implantation was performed in ten patients successfully without any severe complications. Valve functions were perfect in six of ten patients, while the others had insignificant regurgitation by echocardiographic examination at the end of 6 months follow-up.

This study showed that Pulsta® transcatheter heart valve is a feasible, effective, and safe method in the treatment of severe pulmonary regurgitation due to transannular patch repair in patients with tetralogy of fallot.

Right ventricular dysfunction in patients with tetralogy of Fallot and significant pulmonary regurgitation may lead to systolic dysfunction of the left ventricle due to altered ventricular interaction. We were interested in determining whether chronic pulmonary regurgitation affects the preload of the left ventricle. In addition, we wanted to study whether severe chronic pulmonary regurgitation would alter the preload of the left ventricle when compared with patients having preserved pulmonary valve annulus.

The study group comprised 38 patients with tetralogy of Fallot who underwent surgical repair between 1990 and 2003. Transannular patching was required in 21 patients to reconstruct the right ventricular outflow tract. Altogether, 48 age- and gender-matched healthy volunteers were recruited. Cardiac MRI was performed on all study patients to assess the atrial and ventricular volumes and function.

Severe pulmonary regurgitation (>30 ml/m2) was present in 13 patients, of whom 11 had a transannular patch, but only two had a preserved pulmonary valve annulus. The ventricular preload volumes from both atria were significantly reduced in patients with severe pulmonary regurgitation, and left ventricular stroke volumes (44.1±4.7 versus 58.9±10.7 ml/m2; p<0.0001) were smaller compared with that in patients with pulmonary regurgitation <30 ml/m2 or in controls.

In patients with tetralogy of Fallot, severe pulmonary regurgitation has a significant effect on volume flow through the left atrium. Reduction in left ventricular preload volume may be an additional factor contributing to left ventricular dysfunction.

Pulmonary balloon valvuloplasty is a safe and effective treatment for children with pulmonary valve stenosis. A few studies evaluate the long-term outcomes of the procedure, particularly the degree of pulmonary regurgitation. We evaluated the outcomes of children >1 year following valvuloplasty for pulmonary valve stenosis.

A retrospective analysis of children with pulmonary valve stenosis following pulmonary balloon valvuloplasty at a single institution was performed. Clinic summaries, catheterisation data, and echocardiographic data were reviewed. Inclusion criteria were isolated pulmonary valve stenosis, age <19 years at the time of intervention, and at least one echocardiogram performed at least 1 year after valvuloplasty.

A total of 53 patients met inclusion criteria. The median age at valvuloplasty was 0.4 years (0.01–10.6 years). The last follow-up was 4.8±2.3 years following valvuloplasty. The pre-valvuloplasty peak instantaneous gradient by echocardiography was 60.6±14.6 mmHg. The peak gradient at the first postoperative echocardiography was reduced to 25.5±12 mmHg (p<0.001), and further decreased to 14.8±15.8 mmHg (p<0.001) at the most recent follow-up. The degree of regurgitation increased from before valvuloplasty to after valvuloplasty (p<0.001) but did not progress at the most recent follow-up (p=0.17). Only three patients (5.7%) required re-intervention for increasing pulmonary stenosis (two surgical; one repeat balloon). No significant procedural complications occurred.

Pulmonary balloon valvuloplasty remains a safe and effective treatment for children with isolated pulmonary valve stenosis, with excellent long-term outcomes and no mortality. A few patients require further intervention. Long-term follow-up demonstrates decreased, residual stenosis. Patients have a small, acute increase in pulmonary regurgitation following valvuloplasty, but no long-term progression.

At present, the exclusion for percutaneous pulmonary valve implantation for free pulmonary regurgitation after tetralogy of Fallot repair includes an unfavourably large right ventricular outflow tract.

To report feasibility and early experience with a recently developed transcatheter heart valve, Venus P-valveTM, implanted in six patients with severe pulmonary regurgitation with large right ventricular outflow tracts.

There were two female patients and four male patients. The median age of the patients was 18.5 years, and the mean body weight was 53.8 kg. All the patients were in NYHA class II and had severe pulmonary regurgitation after previous transannular patch repair of tetralogy of Fallot. The median time after the last surgical operation was 13.5 years.

The Venus P-valveTM was successfully implanted in all the patients with implanted valve diameters ranging from 24 to 32 mm. The mean fluoroscopy time was 29.8 minutes. None of the patients had significant outflow tract gradient or pulmonary regurgitation immediately after valve implantation. Only one patient had unexpected mild proximal valve migration to the right ventricular body during withdrawal of the delivery system. It caused mild paravalvar leak and significant tricuspid regurgitation. At 6 months follow-up, the median of right ventricular end-diastolic volume indices decreased from 146 to 108 ml/m2 (p-value=0.046). The Doppler systolic peak gradient across the valve ranged from 4 to 40 mmHg, and there was no evidence of stent fracture on fluoroscopy or structural valve failure.

The Venus P-valveTM can be implanted successfully and effectively in patients with severe pulmonary regurgitation and a large right ventricular outflow tract. The early results with this valve are encouraging.