Sixty seven patients with PA-IVS were born in Sweden during the study period and were reviewed for inclusion. For a comprehensive understanding of the assessment of prerequisites for biventricular circulation or univentricular palliation in the two different pediatric thoracic centers, see Tables 1 and 2. 34 patients (11 females) were included in the study. 18 patients (4 females) were initially treated with catheter-based treatment (group A) and 16 (7 females) were initially treated with surgical decompression of the right ventricle (group B). Three patients were intended for catheter-based treatment by mechanical perforation but had a failed valve perforation. In the analyses they were included in group B.
Table 1 Patient characteristics and echocardiogram measurements preoperative for patients whose right ventricle was not decompressedTable 2 Patient characteristics and echocardiogram measurements preoperative for patients whose right ventricle was decompressedAll included patients had a membranous atresia and a bi- or tripartite right ventricle. In group A and B the preoperative tricuspid valve (TV) z-score median was − 0.22 (− 0.66, 1.18) and 0.61 (− 0.03, 1.17) respectively. Preprocedural echocardiographic measurements are presented in Table 2. Three patients in group B had VCACs, no patient had RVDCC. Five patients had tricuspid valve dysplasia, three in group A and two in group B.
There were few associated extracardiac malformations or genetic disorders. Two patients had trisomy 21. One patient had undescended testicle, one patient had hip joint luxation, one patient had cortisol deficiency.
Interventional TechniqueIn group A the initial intervention consisted of a catheter-based valvotomy. Followed by a balloon dilation of the valve. No patient received additional source of pulmonary blood flow placed during the initial intervention.
In group B all patients underwent surgical valvulotomy. Additionally, during the initial intervention five patients underwent atrioseptostomy, one patient had a fenestrated patch placed in the ASD. Nine patients had a modified Blalock-Taussig shunt (mBTS), three patients had a transannular patch repair, two patients had a homograft to replace the pulmonary valve, one patient had a dilation of the pulmonary bifurcation, one patient had the PDA ligated.
OutcomesThere was no mortality in either group during the entire study period. One patient was lost to follow-up after discharge. Excluding this patient, follow-up time ranged from 2 to 15 years (median 9 years, 6, 13).
In group A, all attempted perforations with radiofrequency of the pulmonary valve were successful. Two out of five of the attempted mechanical perforations were successful. The 3 patients that had a failed mechanical perforation were included in, and their outcome was analyzed with, group B. 16 out of 18 patients reached biventricular circulation as the end result while 2 patients had a one-and-a half ventricle (1,5-ventrice) repair (open pulmonary valve and bidirectional Glenn anastomosis) at the end of the study. In group B, 15 out of 16 patients reached biventricular circulation while one patient required univentricular palliation.
In group A, 30 days after their initial intervention eight patients had no additional pulmonary blood flow and ten patients had additional pulmonary blood flow through either a mBTS or a PDA-stent, see Table 3. In group B 30 days after their initial intervention four patients had no additional pulmonary blood flow and twelve patients had additional pulmonary blood flow through a mBTS. Four patients with tricuspid valve dysplasia achieved biventricular circulation (3 in group A and one in group B), one patient in group B ended up with univentricular circulation.
Table 3 Reinterventions, outcomes, and complications 30 days after initial interventionOne patient in group B was lost to follow-up after hospital discharge due to living abroad.
Length of Hospital StayPatients in group A had a tendency towards fewer days in the intensive care unit during their initial procedural admission, i.e., when they had their initial intervention, compared to patients in group B, median 3 (0, 11) and 7 (3, 17) days, respectively (p-value 0.11). Seven patients in group A were not admitted to the intensive care unit at all. The patients in group B exhibited a tendency towards a shorter overall hospital stay compared to group A, median 18 (10, 20) and 21 (15, 36) days, respectively (p-value 0.078).
ReinterventionsGroup A16 out of 18 patients in group A had at least one reintervention, see Fig. 1. Ten patients had a reintervention within 30 days after their initial intervention, five had a re-catheterization where they received a PDA-stent and five patients underwent heart surgery to facilitate additional pulmonary blood flow, see Table 3. 14 patients underwent at least one re-catheterization. Eleven patients underwent heart surgery after the initial catheterization. Nine patients had moderate-severe tricuspid regurgitation (TR) preoperatively, three of these patients needed additional pulmonary blood flow after initial catheterization, none of the patients that needed a reintervention within 30 days after initial intervention had tricuspid valve dysplasia. During long-term follow-up (after the initial procedural admission), there were in total 24 catheter-based reinterventions and 16 surgical.
Fig. 1Overview of reinterventions
Group BEleven out of 16 patients in group B had at least one reintervention, of whom four patients had a reintervention within 30 days after the initial intervention, all of which were surgical, see Table 3. Nine patients received a mBTS in their first intervention in addition to the surgical valvulotomy. Six patients had at least one catheter-based intervention. Ten patients had at least one reoperation. Six patients had moderate-severe TR preoperatively, one of whom required reoperation within 30 days after their first surgery, this patient had tricuspid valve dysplasia. During long-term follow-up, there were in total nine catheter-based reinterventions and 14 surgical.
ComplicationsIn group A, one patient experienced a periprocedural complication, a right ventricle perforation and atrial flutter during catheterization, this resolved spontaneously, the patient was discharged after 14 days. Furthermore, within 30 days after their initial intervention, six patients experienced complications, including three shunt occlusions in arterial pulmonary shunts (AP-shunts) (one of which led to circulatory collapse and brain hemorrhage) one case of cerebral infarction, one case of necrotizing enterocolitis and acute kidney failure, and one case of infection. The patients who received a PDA-stent had no complications related to the stents.
In group B, three patients had a failed balloon valvulotomy before undergoing heart surgery. Within 30 days after the initial intervention, three patients in group B experienced complications: one patient had diaphragm paresis, one patient had anuria and needed dialysis, one patient had a stroke, suffered arrythmia, had a deep vein thrombosis, needed dialysis and ECMO and also suffered necrosis of fingers and toes.
Systematic Review of Current Literature422 articles were identified, after duplicates were removed 414 articles remained and their abstracts were screened for relevance, see Fig. 2. All studies were assessed in regard to inclusion criteria and after that 25 studies were included in the review. Partially the same patient population were studied in two studies [10, 11]. In order to avoid analyzing duplicate results, data have been collected from all studies but each variable from each patient population was only analyzed once, see Table 4. The objectives, settings, inclusion criteria and z-score models of the included studies differed, and a meta-analysis could therefore not be performed.
Fig. 2Systematic literature search according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)—guidelines
Table 4 Summary of systematic literature reviewPatient CharacteristicsThe inclusion criterion in several studies was based on TV z-score, it ranged from > − 5 to > − 1 [9, 11, 12, 14, 17, 29]. All patients were considered to have a favorable morphology of the right ventricle, suitable for biventricular repair. A few patients had a muscular atresia or a hypoplastic right ventricle [11, 23]. tricuspid valve dysplasia was an exclusion criterion in several studies [14, 16].
Intervention ApproachesThe included studies evaluated different intervention approaches including surgical decompression of the right ventricle, hybrid interventions, radiofrequency perforation with subsequent balloon dilation of the pulmonary valve, and various mechanical perforation techniques using chronic total occlusion (CTO)-wires and coronary guidewires. Different studies have investigated the possibility to use the soft or the stiff end of different coronary wires and if a retrograde approach instead of the antegrade, which is standard in most centers, can have benefits [2, 6, 11, 12, 14, 15, 17, 18, 20, 25, 26]. The hybrid approach has emerged as an alternative to percutaneous treatment, aiming to reduce the perforation risk and enabling the placement of a mBTS if necessary without requiring additional interventions. The technique involves a midline sternotomy and under transesophageal echocardiographic guidance the pericardium and pulmonary valve is perforated with a needle and balloon pulmonary valvuloplasty is then performed [16, 22,23,24, 30]. Cardiopulmonary bypass (CPB) is avoided and the ductus arteriosus can be snared, if the patient then desaturates a stent in the ductus or a mBTS can be placed.
OutcomesSuccess Rate and MortalityThe success rate of catheter-based intervention in perforating the pulmonary valve ranged between 74 and 100% [2, 6,7,8,9,10, 12,13,14,15, 17, 18, 20, 25, 26, 29] and for hybrid approach 86–100% [16, 22,23,24, 28, 30, 31], see Table 4. Failed intervention was correlated to lower age, weight and BSA [10]. The early mortality (death within 30 days post procedure or death during the first hospitalization) varied from 0 to 44.1% across studies (hybrid procedure 0–9%, catheter-based procedure 0–44.1%, surgical procedure 0–12%) [10, 12, 14,15,16,17, 19, 21, 24, 25, 29]. The leading cause of death in several studies was infection or sepsis [11, 15, 24, 25, 29]. Prolonged stays in the PICU were found to be associated with higher mortality rates, even if the patients initially survived the intervention.
The length of follow-up varied among the included studies, ranging from no follow-up after discharge from hospital to 5.4 years [6, 7, 9, 11,12,13,14,15,16,17,18,19,20,21,22,23,24,25, 29, 30].
Predictors and Likelihood for Biventricular Circulation or ReinterventionSeveral studies have focused on identifying predictors of biventricular circulation and the likelihood of reintervention or need for additional pulmonary blood flow [7, 9, 19, 22, 24,
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