The France PCI registry: Design, methodology and key findings.

BACKGROUND: Obstructive coronary artery disease is the main cause of death worldwide. By tracking events and gaining feedback on patient management, the most relevant information is provided to public health services to further improve prognosis. AIMS: To create an inclusive and accurate registry of all percutaneous coronary intervention (PCI) procedures performed in France, to assess and improve the quality of care and create research incentives. Also, to describe the methodology of this French national registry of interventional cardiology, and present early key findings. METHODS: The France PCI registry is a multicentre observational registry that includes consecutive patients undergoing coronary angiography and/or PCI. The registry was set up to provide online data analysis and structured reports of PCI activity, including process of care measures and assessment of risk-adjusted outcomes in all French PCI centres that are willing to participate. More than 150 baseline data items, describing demographic status, PCI indications and techniques, and in-hospital and 1-year outcomes, are captured into local reporting software by medical doctors and local research technicians, with subsequent encryption and internet transfer to central data servers. Annual activity reports and scoring tools available on the France PCI website enable users to benchmark and improve clinical practices. External validation and consistency assessments are performed, with feedback of data completeness to centres. RESULTS: Between 01 January 2014 and 31 December 2022, participating centres increased from six to 47, and collected 364,770 invasive coronary angiograms and 176,030 PCIs, including 54,049 non-ST-segment elevation myocardial infarction cases and 31,631 ST-segment elevation myocardial infarction cases. Fifteen studies stemming from the France PCI registry have already been published. CONCLUSIONS: This fully electronic, daily updated, high-quality, low-cost, national registry is sustainable, and is now expanding. Merging with medicoeconomic databases and nested randomized scientific studies are ongoing steps to expand its scientific potential.

Early survival after acute myocardial infarction with ST-segment elevation: What could be improved? Insights from France PCI French registry.

Early mortality post-ST-segment elevation myocardial infarction (STEMI) in France remains high. The multicentre France Percutaneous Coronary Intervention Registry includes every patient undergoing coronary angiography in France. We analyzed the prevalence and impact of unmodifiable and modifiable risk factors on 30-day survival in patients experiencing STEMI. Patients admitted for STEMI between 01/2014 and 12/2016 were included in the analysis. Patients with nonobstructive coronary artery disease, with cardiogenic shock or cardiac arrest without STEMI, were excluded. Prehospital, clinical and procedural data were collected prospectively by the cardiologist in the cath lab using medical reporting software. Information on outcomes, including mortality, was obtained by a dedicated research technician by phone calls or from medical records. Marginal Cox proportional hazards regression was used to test the predictive value for survival at 30 days in a multivariable analysis. Included were 2590 patients (74% men) aged 63 ± 14 years. During the first month, 174 patients (6.7%) died. After adjustment, unmodifiable variables significantly associated with reduced 30-day survival were: age > 80 years (prevalence 15%; hazard ratio [HR] 2.7; 95% confidence interval [CI] 1.5-4.7), chronic kidney disease (2%; HR 5.3; 95% CI 2.6-11.1), diabetes mellitus (14%; HR 1.6; 95% CI 1.0-2.5), anterior or circumferential electrical localization (39%; HR 2.0; 95% CI 1.4-2.9), and Killip class 2, 3, or 4 (7%; HR 3.4; 95% CI 1.9-5.9; 2%; HR 10.1; 95% CI 5.3-19.4; 4%; HR 18; 95% CI 10.8-29.8, respectively). Among modifiable variables, total ischemic time > 3 hours (68%; HR 1.8; 95% CI 1.1-3.0), lack of appropriate premedication (18%; HR 2.2; 95% CI 1.5-3.3), and post-PCI TIMI < 3 (6%; HR 4.9; 95% CI 3.2-7.6) were significantly associated with reduced 30-day survival. Most predictors of 30-day survival post-STEMI are unmodifiable, but outcomes might be improved by optimizing modifiable factors, most importantly ischemic time and appropriate premedication.

Characteristics and Prognosis of Patients With Fibromuscular Dysplasia in a Population of Spontaneous Coronary Artery Dissections (from the French Registry of Spontaneous Coronary Artery Dissections "DISCO").

Spontaneous coronary artery dissection (SCAD) and fibromuscular dysplasia (FMD) are pathologies that appear to be closely related. This study compares the characteristics of the FMD population to the non-FMD population in a SCAD cohort. It thus assesses the involvement of the FMD phenotype in a SCAD population. From the data of the French DISCO registry, we included patients with a diagnosis of SCAD and in whom a search for FMD was performed. We collected the following characteristics of this population: the clinical and angiographic presentation, the data concerning the management, and the events occurring during the follow-up. In the 373 SCADs confirmed in the DISCO registry, we obtained imaging data for 340 of them. FMD was found in 45% of cases. The mean age was higher in the FMD group, 53.2 ± 8.8 years, versus 50.1 ± 11 years in the non-FMD group. High blood pressure and postmenopausal status were significantly higher in the FMD group. Clinical presentation, angiographic data, and management were comparable. The major adverse cardiac event rate and recurrence rate were not different between the 2 groups after 1 year of follow-up. In conclusion, we confirmed a 45% prevalence of FMD in the SCAD population. The median age was higher in the FMD group, suggesting that FMD may develop over time. The rate of major adverse cardiac events and recurrence were similar in the FMD group versus the non-FMD group after 1 year of follow-up.

Pulmonary Hypertension in Patients with Common Variable Immunodeficiency.

PURPOSE: Common variable immunodeficiency (CVID) is known to cause infectious, inflammatory, and autoimmune manifestations. Pulmonary hypertension (PH) is an unusual complication of CVID with largely unknown characteristics and mechanisms. METHODS: We report the clinical, functional, hemodynamics, radiologic and histologic characteristics, and outcomes of CVID-associated PH patients from the French PH Network. RESULTS: Ten patients were identified. The median (range) age at CVID diagnosis was 36.5 (4-49) years and the median delay between CVID and PH diagnosis was 12 (0-30) years. CVID-associated PH affected predominantly women (female-to-male ratio 9:1). Most patients were New York Heart Association functional class III with a severe hemodynamic profile and frequent portal hypertension (n = 6). Pulmonary function tests were almost normal in 70% of patients and showed a mild restrictive syndrome in 30% of patients while the diffusing capacity for carbon monoxide was decreased in all but one patient. High-resolution computed tomography found enlarged mediastinal nodes, mild interstitial infiltration with reticulations and nodules. Two patients had a CIVD-interstitial lung disease, and one presented with bronchiectasis. Pathologic assessment of lymph nodes performed in 5 patients revealed the presence of granulomas (n = 5) and follicular lymphoid hyperplasia (n = 3). At last follow-up (median 24.5 months), 9 patients were alive, and one patient died of Hodgkin disease. CONCLUSION: PH is a possible complication of CVID whose pathophysiological mechanisms, while still unclear, would be due to the inflammatory nature of CVID. CVID-associated PH presents as precapillary PH with multiple possible causes, acting in concert in some patients: a portal hypertension, a pulmonary vascular remodeling, sometimes a pulmonary parenchymal involvement and occasionally an extrinsic compression by mediastinal lymphadenopathies, which would be consistent with its classification in group 5 of the current PH classification.

Use and outcomes of the PK Papyrus covered stent in France: SOS PK Papyrus Registry.

OBJECTIVES: To evaluate the rate of procedural success and long-term outcomes of the PK Papyrus (PKP) covered stent (CS). BACKGROUND: CS are essential in the treatment of coronary artery perforation (CAP). They have also been used to treat coronary artery aneurysms. Limited evidence is available on clinical outcomes with the PKP. METHODS: This was a multicenter, observational, retrospective, and prospective study. Consecutive patients undergoing intentional PKP implantation in 22 centers in France were included. The primary endpoint was the rate of procedural success. Secondary endpoints included rates of death, myocardial infarction (MI), target lesion revascularization (TLR), in-stent restenosis (ISR), and stent thrombosis (ST). RESULTS: Data from 130 patients were analyzed (mean age 72.5 ± 10.5 years; 71% men). The main indication for PKP was CAP, in 84 patients (65%). Delivery success was achieved in 95% and procedural success in 91%. During the in-hospital stay, 15 patients died (12%) and 7 (5%) presented with ST. Data from 127 patients were available at 19.2 ± 12.8 month follow-up. Thirty-three patients died (26%), 15 (12%) had an MI and 21 (17%) presented with TLR. TLR was due to ISR in 12 patients (9%), 10 had definite ST (8%) and 1 patient for stent under-expansion. CONCLUSIONS: The principal indication for PKP was CAP. PKP had high rates of delivery and procedural success. At long-term follow-up, there was a high rate of TLR, mainly due to ISR and ST. These results are consistent with previously reported data in these clinical settings.

Incidence, delays, and outcomes of STEMI during COVID-19 outbreak: Analysis from the France PCI registry.

Objectives: The aim of this study was to assess the impact of the coronavirus disease 2019 (COVID-19) outbreak on incidence, delays, and outcomes of ST-elevation myocardial infarction (STEMI) patients undergoing primary percutaneous coronary intervention (PPCI) in France. Methods: We analyzed all patients undergoing PPCI <24 hours STEMI included in the prospective France PCI registry. The 2 groups were compared on mean monthly number of patients, delays in the pathway care, and in-hospital major adverse cardiac events (MACE: death, stent thrombosis, myocardial infarction, unplanned coronary revascularization, stroke, and major bleeding). Results: From January 15, 2019 to April 14, 2020, 2064 STEMI patients undergoing PPCI were included: 1942 in the prelockdown group and 122 in the lockdown group. Only 2 cases in the lockdown group were positive for COVID-19. A significant drop (12%) in mean number of STEMI/month was observed in the lockdown group compared with prelockdown (139 vs 122, P < 0.04). A significant increase in "symptom onset to first medical contact" delay was found for patients who presented directly to the emergency department (ED) (238 minutes vs 450 minutes; P = 0.04). There were higher rates of in-hospital MACE (7.7% vs 12.3%; P = 0.06) and mortality (4.9% vs 8.2%; P = 0.11) in the lockdown group but the differences were not significant. Conclusion: According to the multicenter France PCI registry, the COVID-19 outbreak in France was associated with a significant decline in STEMI undergoing PPCI and longer transfer time for patients who presented directly to the ED. Mortality rates doubled, but the difference was not statistically significant.

Phenotype and outcome of pulmonary arterial hypertension patients carrying a TBX4 mutation.

INTRODUCTION: TBX4 mutation causes small patella syndrome (SPS) and/or pulmonary arterial hypertension (PAH). The characteristics and outcomes of PAH associated with TBX4 mutations are largely unknown. METHODS: We report the clinical, functional, radiologic, histologic and haemodynamic characteristics and outcomes of heritable PAH patients carrying a TBX4 mutation from the French pulmonary hypertension (PH) network. RESULTS: 20 patients were identified in 17 families. They were characterised by a median age at diagnosis of 29 years (0-76 years) and a female to male ratio of three. Most of the patients (70%) were in New York Heart Association (NYHA) functional class III or IV with a severe haemodynamic impairment (median pulmonary vascular resistance (PVR) of 13.6 (6.2-41.8) Wood units). Skeletal signs of SPS were present in 80% of cases. Half of the patients had mild restrictive or obstructive limitation and diffusing capacity of the lung for carbon monoxide (D LCO) was decreased in all patients. High-resolution computed tomography (HRCT) showed bronchial abnormalities, peri-bronchial cysts, mosaic distribution and mediastinal lymphadenopathies. PAH therapy was associated with significant clinical improvement. At follow-up (median 76 months), two patients had died and two had undergone lung transplantation. One-year, three-year and five-year event-free survival rates were 100%, 94% and 83%, respectively. Histologic examination of explanted lungs revealed alveolar growth abnormalities, major pulmonary vascular remodelling similar to that observed in idiopathic pulmonary arterial hypertension (IPAH) and accumulation of cholesterol crystals within the lung parenchyma. CONCLUSION: PAH due to TBX4 mutations may occur with or without skeletal abnormalities across a broad age range from birth to late adulthood. PAH is usually severe and associated with bronchial and parenchymal abnormalities.