[Secondary hypertension: diagnosis and treatment]. / Ipertensione arteriosa secondaria: diagnosi e trattamento.

Hypertension does not recognize obvious pathogenic causes in the majority of patients (essential hypertension). However, a secondary underlying cause of hypertension can be recognized in 5-10% of unselected hypertensive patients, and this prevalence may increase to more than 20% in patients with hypertension that is difficult to control or frankly resistant to treatment. In children, secondary hypertension is most often due to aortic coarctation, distal thoracic or abdominal aortic stenosis, or specific gene mutations. In adults or elderly individuals, secondary hypertension is most often due to atherosclerotic renal artery stenosis, primary hyperaldosteronism, and Cushing's disease or syndrome. Parenchymal nephropathy and hyperparathyroidism can cause hypertension at all ages, while pheochromocytoma and paraganglioma tend to occur more often in adolescents or young adults. In general, secondary hypertension should be suspected in subjects with: (a) onset of hypertension under 30 years of age especially if in the absence of hypertensive family history or other risk factors for hypertension; (b) treatment-resistant hypertension; c) severe hypertension (>180/110 mmHg), malignancy, or hypertensive emergencies; d) rapid rise in blood pressure values in previously well controlled patients. Any clinical signs suspicious or suggestive of hypertension from endocrine causes, a "reverse dipping" or "non-dipping'" profile at 24 h ambulatory blood pressure monitoring not justified by other factors, signs of obvious organ damage may be helpful clues for diagnosis. Finally, patients snoring or with clear sleep apnea should also be considered for possible secondary hypertension.

Evaluation of short and mid-term clinical outcomes in patients with aortic coarctation treated with self-expandable stents.

The present study aimed to evaluate the outcomes of percutaneous treatment of aortic coarctation using self-expandable uncovered Nitinol stents. We conducted a retrospective clinical data review of all patients with aortic coarctation and treated with self-expandable uncovered Nitinol stents at our institution between 2009 and 2019. The gradient pressure across the coarctation site was measured using aortography. Follow-up echocardiography and computed tomography angiography were performed to assess possible stent complications. A total of 127 stents were successfully implanted in 125 patients (64.8% males) with a mean age of 35.36 ± 11.9 years. The gradient across the coarctation site decreased significantly from 67.48 ± 14.79 to 5.04 ± 3.01 mmHg (P < 0.001) after self-expandable stent implantation. Systolic blood pressure (SBP) decreased significantly from 175.53 ± 15.99 to 147.22 ± 12.83 mmHg (P < 0.001) after self-expandable stenting. There were no major technical or clinical complications, including balloon rupture, aneurysmal formation, infection, secondary stent migration, thrombosis, death during the procedure, and in-hospital mortality. On a mean follow-up of 48 ± 23.6 months (12-120 months), the gradient [from 59.43 ± 15.42 to 3.72 ± 1.38 mmHg (P < 0.001)] and SBP [from 175.53 ± 15.99 to 127.99 ± 7.82 mmHg (P < 0.001)] decreased significantly. There was no mortality, aneurysmal formation in the stent site, dislocation, or aortic re-stenosis requiring intervention during mid-term follow-up. Treatment of aortic coarctation using a self-expandable uncovered nitinol stent is safe and effective with promising mid-term outcomes.

Results of the multicenter early feasibility study (EFS) of the Renata Minima stent as treatment for branch pulmonary artery stenosis and coarctation of aorta in infants.

BACKGROUND: Stent implantation has become standard of care in older children and adults for treatment of branch pulmonary artery stenosis (BPAS) and coarctation aorta (CoAo). There are no stents approved or available for infants that have the potential to be dilated to adult diameters. The Minima stent was designed to fulfill this unmet need. METHODS: Multicenter, prospective, nonrandomized early feasibility study evaluating safety and effectiveness of the Minima stent for treatment of BPAS and CoAo. Primary endpoints included: (1) successful deployment across lesion, (2) stenosis relief defined by an increase in angiographic diameter of >50% and (3) freedom from stent explant, embolization or migration at 30 days and 6 months. RESULTS: Between 2/2022 and 5/2022, 10 pts underwent Minima stent implantation with a median age and weight of 9 months (4-43 months) and 7.6 kg (5.1-16.9 kg). Procedural success and predefined stenosis relief was achieved in all cases (CoAo [n = 4], BPAS [n = 6]). Adverse events occurred in 3 pts: transient diminished lower extremity pulse (n = 2), distal stent on-balloon displacement successfully managed in the catheterization suite (n = 1). There were no deaths or major adverse events. All patients were free from stent explant and migration at 30 days and 6 months with no evidence for significant restenosis at latest follow-up. CONCLUSIONS: Implantation of the Renata Minima stent was safe and effective for the treatment of BPAS and CoAo in this small cohort of infants and young children during early follow-up. Based on these early results, an expanded study with longer follow-up is warranted.

Follow-up outcomes and effectiveness of stent implantation for aortic coarctation: A systematic review and meta-analysis.

BACKGROUND: The authors aim to investigate the clinical implications of stent use for the management of CoA. METHODS: All observational studies on stent implantation for the treatment of aortic coarctation and the relevant RCTs were systematically retrieved. Outcomes included the immediate success rate, pre- and post-stent gradient, survival, minor and major complications, restenosis, post-stent systolic blood pressure, and reintervention rate. The analysis was further stratified by CoA type, stent type, and the mean age of the patients. RESULTS: Our meta-analysis incorporated 66 eligible studies involving 3,880 patients. The success rates for stent placement, defined as achieving post-treatment gradients of ≤20 mmHg and 10 mmHg, 0.96 (95% CI: 0.95 - 0.97; I2 = 59.83%) and 0.92 (95% CI: 0.89 - 0.95, I2 = 77.63%) respectively. The complication rates were quite low, with minor and major complication rates of 0.017 (95% CI: 0.013 - 0.021) and 0.007 (95% CI: 0.005 - 0.009), respectively. Unplanned reinterventions were required at a rate of 0.021 (95% CI: 0.015 - 0.026). At a mean follow-up of 2.9 years, 97% of the patients survived and 28% remained on antihypertensive therapy. While immediate effectiveness was consistent across age groups, complications were more prevalent in patients aged <20 years, and long-term efficacy was lower in those aged >20 years. Encouragingly, in neonates and infants, CoA stenting yielded results comparable to those observed in older children. CONCLUSION: These findings underscore the overall favorable outcomes of stent placement for aortic coarctation, with considerations for age-related variations in complications and long-term efficacy.

Transcatheter management of acquired atresia of aorta.

A 24-year-old man was referred to our tertiary care center for the management of uncontrolled hypertension secondary to severe coarctation of aorta.

Long-term follow-up after endovascular treatment of aortic coarctation with bare and covered Cheatham platinum stents.

BACKGROUND: Endovascular treatment of aortic coarctation (CoA) in children and adults frequently requires stent implantation. The aim of this study was to analyze long-term results after CoA treatment with bare and covered Cheatham-PlatinumTM (CP) stents in our institution and to derive recommendations for the differential use of these stent types. METHODS: In this retrospective single institution study, 212 patients received endovascular CoA treatment with bare (n = 71) and covered (n = 141) CP stents between September 1999 and July 2021, respectively. The indications for treatment were native CoA in 110/212 patients (51.9%) and re-coarctation after primary surgical or interventional treatment in 102/212 patients (48.1%). Median patient age at endovascular CoA treatment was 18.8 years [IQR 11.9; 35.8]. Long-term follow-up was available in 158/212 patients (74.5%) with a median follow-up of 7.3 years [IQR 4.3; 12.6]. RESULTS: Procedural success was achieved in 187/212 (88.2%) patients. Survival rate was 98.1% after 5, and 95.6% after 10 and 15 years, respectively. The probability of freedom from re-intervention was 93.0% after 5, 82.3% after 10 and 77.8% after 15 years, respectively. Freedom from re-interventions (44/158, 27.8%) did not differ between patients who received bare or covered CP stents (p = 0.715). Multivariable risk factor analysis identified previous CoA surgery (HR: 2.0, 95% confidence interval (CI): 1.1-3,9, p = 0.029), postdilatation (HR: 2,9, 95% CI: 1.1-6.3, p = 0.028) and age at intervention (HR: 0.96, 95% CI: 0.94-0.99, p = 0.002) as independent risk factors for re-intervention. Peri-procedural complications occurred in 15/212 (7.1%) patients (dissection/thrombosis of vascular access vessel: n = 9; bleeding: n = 1; stent dislocation: n = 2; aortic dissection/aortic wall rupture: n = 3). Long-term complications were observed in 36 patients and included stent fracture (n = 19), aneurysm formation (n = 14), endoleak (n = 1) and subclavian artery stenosis (n = 2). Peri-procedural and long-term complications did not differ between patients who received CoA treatment with bare or covered CP stents (all p > 0.05). CONCLUSION: Endovascular treatment of CoA using bare or covered CP stents can be performed safely and effectively with excellent long-term results. Survival, re-intervention and complication rate did not significantly differ between both stent types. However, individual stent selection is advisable with regard to CoA morphology and severity as well as patient age.

Evolving Utilization of Covered Stents for Treatment of Aortic Coarctation: Report From the IMPACT Registry.

BACKGROUND: Covered stent (CS) use for the transcatheter treatment of coarctation of the aorta (COA) was first described over 20 years ago. The covered Cheatham-platinum stent received Food and Drug Administration approval for COA treatment in 2016. Data collected as part of the National Cardiovascular Data Registry IMPACT registry were evaluated for contemporary usage patterns of CS for the treatment of COA from 2016 to 2021. METHODS: Version 2 of the IMPACT registry was queried for all patients who underwent stent placement for treatment of COA from 2016 to 2021. Trends in CS use were evaluated by year and age of patient at the time of implant. Analysis was limited to clinical factors collected as part of the registry and was performed to identify factors associated with CS use. RESULTS: Data were available for 1989 case entries. Most patients (92%) received a single stent. The overall percentage of CS use in the cohort was 23% and remained consistent throughout the study period. The probability of CS use was significantly associated with increasing patient age at implant. Additional factors associated with CS use included smaller initial COA diameter, native COA, and the presence of a pseudoaneurysm. Procedural adverse event rates were low. CONCLUSIONS: CS use to treat COA was more common in adult patients and remained stable over the study period. Factors associated with CS use, including smaller COA diameter and aortic pseudoaneurysm, demonstrate the perceived value of CS as a tool to reduce the risk of aortic wall injury during treatment of COA.

Outcomes of Heart Failure Hospitalizations in Adult Patients With Coarctation of Aorta: Report From National Inpatient Sample.

Coarctation of aorta (CoA) is a common congenital anomaly which portends patients to early diastolic and systolic heart failure. In this retrospective cohort study, we aimed to evaluate the impact of CoA on heart failure hospitalization. Using the national inpatient sample, the study compared the outcomes of heart failure hospitalization between patients with and without CoA. We noted increasing prevalence of CoA related heart failure admissions over the last decade. Heart failure patients with CoA were younger (mean age 57 vs 71.6 years, P < 0.001), had a longer length of stay (7.4 vs 5.4 days, P < 0.001), and a higher incidence of cardiogenic shock (6.5% vs 2.1%, P = 0.001). However, there was no statistically significant difference in in-hospital mortality (OR 1.45, 95% CI: 0.58, 3.62, P = 0.421) between both groups. These findings demonstrate that CoA increase healthcare resource utilization in patients admitted with heart failure without any significant increase in in-hospital mortality.

Cardiovascular considerations in management of patients with Turner syndrome.

Turner syndrome (TS) is a chromosomal disorder that affects 25-50 per 100,000 live born females. Patients with TS face a heavy burden of cardiovascular disease (congenital and acquired) with an increased risk of mortality and morbidity compared to the general population. Cardiovascular diseases are a major cause of death in females with TS. Approximately 50% of TS patients have a congenital heart abnormality, with a high incidence of bicuspid aortic valve, coarctation of the aorta and generalised arteriopathy. Frequently, females with TS have systemic hypertension, which is also a risk factor for progressive cardiac dysfunction and aortopathy. This paper aims to provide an overview of the cardiovascular assessment, management and follow up strategies in this high-risk population.

A new FDA approved stent for congenital heart disease: First-in-man experiences with G-ARMORTM.

We present the first clinical experience with a new hybrid cell structure covered stent, designed for congenital heart disease applications. It represents a significant redesign of the Cheatham Platinum (CP) Stent (Numed Inc.), maintaining the traditional benefits of the covered CP whilst significantly decreasing shortening and allowing controlled flaring at the ends through its combination of larger and standard sized cells. We first implanted the stent in 2 patients with superior sinus venosus defects with anomalous drainage of the right upper and middle lobe pulmonary veins. The first was a 40 year male and the second a 36 year old female. The third case was a 60 year old patient with near atresia of the aorta, with pre and poststenotic aortic dilation. The clinical result in all cases was excellent with no obstruction to pulmonary venous return and no visible L-R shunt on the transthoracic echo on 24 h and 2 week follow-up for the patient with sinus venosus defects and uniform complete revascularization of the aorta without any vascular complications in the patient with coarctation. These are the first uses of this stent in human subjects. The design is specifically aimed toward procedures where stent shortening is undesirable. Hence, coarctation of the aorta as well as stent implantation in preparation for percutaneous pulmonary valve placement are obvious use areas, as well as the growing body of evidence supporting percutaneous treatment of sinus venosus defects.

Mid-aortic syndrome: a rare cause of heart failure in infants.

This case reports describe a rare disease, mid-aortic syndrome (MAS), that can cause severe heart failure and hypertension in infancy. The typical images, key points of diagnosis, and therapy methods of the disease have also been presented. We report two critical thoracoabdominal aortic coarctation cases in infants aged 2 and 11 months with severe heart failure. The patients were initially misdiagnosed as dilated myocardiopathy, with the correct diagnosis confirmed through imaging. Both patients underwent balloon angioplasty; one patient also had bare-metal stents implanted. The patient treated with balloon angioplasty alone died after the procedure, whereas the other patient recovered well. In conclusion, careful physical examinations, especially upper and lower extremity blood pressure differences and palpation of upper and lower limb pulses, are critical in unexplained infant heart failure cases. Stent implantation may be a safer and more effective treatment than simple balloon angioplasty in infants with MAS.

Coarctation of the aorta: Prenatal assessment, postnatal management and neonatal outcomes.

Coarctation of the aorta (Coa) is a potentially life threatening diagnosis. It occurs in 0.3 per 1000 live births and accounts for 6-8% of all infants with congenital heart defects. Neonates with severe Coa may be completely asymptomatic at birth, as the ductus arteriosus can provide flow to the lower body. Those who are not diagnosed prenatally may be diagnosed only after constriction of the ductus arteriosus, when they present in cardiogenic shock. This group has a higher risk for mortality and morbidity relative to those diagnosed prenatally. Despite the increasing practice of universal pulse oximetry screening, many cases with significant coarctation of the aorta still go undiagnosed in the newborn period. In this article, we present the pathophysiology, diagnosis, presentation, treatment and outcomes of Coa.

Balloon-Expandable Cheatham-Platinum Stents Versus Self-Expandable Nitinol Stents in Coarctation of Aorta: A Randomized Controlled Trial.

OBJECTIVES: This study sought to compare the safety and efficacy of the balloon-expandable stent (BES) and the self-expandable stent (SES) in the endovascular treatment of coarctation of aorta. BACKGROUND: Coarctoplasty with stents has conferred promising results. Although several nonrandomized studies have approved the safety and efficacy of the BES and the SES, no high-quality evidence exists for this comparison. METHODS: In the present open-label, parallel-group, blinded endpoint randomized pilot clinical trial, adult patients with de novo native aortic coarctation were randomized into Cheatham-platinum BES and uncovered nitinol SES groups. The primary outcome of the study was a composite of procedural and vascular complications. The secondary outcomes of the study consisted of the incidence of aortic recoarctation, thoracic aortic aneurysm/pseudoaneurysm formation, and residual hypertension at a 12-month follow-up. RESULTS: Among 105 patients who were screened between January 2017 and December 2019, 92 eligible patients (32 women [34.8%]) with a median age of 30 years (IQR: 20-36 years) were randomized equally into the BES and SES groups. The composite of procedural and vascular complications occurred in 10.9% of the BES group and 2.2% of the SES group (odds ratio: 0.18; 95% CI: 0.02-1.62; P = 0.20). Aortic recoarctation occurred in 5 patients (5.4%), 3 patients (6.5%) in the BES group and 2 patients (4.3%) in the SES group (odds ratio: 0.65; 95% CI: 0.10-4.09; P = 0.64). Only 1 patient (1.1%) was complicated by aortic pseudoaneurysm. Hypertension control was achieved in 50% of the study population, with an equal distribution in the 2 study groups at the 12-month follow-up. CONCLUSIONS: Both the BES and the SES were safe and effective in the treatment of native coarctation.

Prospective multicenter study of the breakable babystent for treatment of aortic coarctation in newborns and infants.

To assess the efficacy and safety of a breakable BabyStent to treat complex aortic coarctation (CoA) in early childhood. Although recommended in several guidelines, there is no approved aortic stent for young infants, because of the dilemma between two mandatory requirements: expandable up to adult size on the one hand, and small enough to fit through a baby's femoral artery on the other. Prospective interventional, multi-center clinical trial with the breakable Osypka BabyStent® (OBS). The OBS is a low-profile, 15-mm long cobalt-chromium stent, pre-mounted on a 6 mm balloon and inserted via a 4 Fr sheath. After implantation, its diameter is adjustable from 6 to 12 mm by balloon dilation. Further dilation opens predefined joints enabling unrestricted growth. Nineteen patients (9 male), median age 112 days (range: 7-539), median body weight 5.6 kg (range: 2.4-8.4) were deemed high risk and underwent stent implantation. Of those, 74% suffered from re-CoA following surgery, 53% had additional cardiac and 21% noncardiac malformations. Our primary combined endpoint was fulfilled: All stents were implanted in the desired region, and a >50% intrastenotic diameter-extension was achieved in 15 patients (78.9%, 80% confidence interval [62.2; 90.5], 95% confidence interval [54.4; 93.9]). Secondary endpoint confirmed that the OBS fits the baby's femoral vessel diameter. All children survived the procedure and 12-month follow-up. This stent enables percutaneous stenting of complex aortic coarctation to treat high-risk newborns and infants.

Endovascular treatment of aortic coarctation with a novel BeGraft aortic stent in children and young adults: a single-centre experience with short-term follow-up results.

OBJECTIVES: We present our experience and outcomes with the BeGraft in the treatment of aortic coarctation in a predominantly paediatric population. METHODS: This study includes a retrospective analysis of patients who had Begraft aortic stent implantation between 2018 and 2020 from a single centre. RESULTS: The BeGraft aortic stent was used in 11 patients (7 males, 4 females) with a median age of 14 (13-21) years and a median weight of 65 (46-103) kg. Coarctation was native in five patients and recurrent in six patients. Median stent diameter and length were 16 mm and 38 mm, respectively. The median peak-to-peak pressure was 30 (12-55) mmHg before the procedure and 5 (0-17) mmHg after the procedure. The stenting procedure was successful in 10 of the 11 patients. Stent migration to the abdominal aorta occurred on post-procedure day 1 in the 21-year-old patient, who had previously undergone surgical closure of the ventricular septal defect and balloon angioplasty for coarctation. After repositioning failed, the stent was safely fixed in the abdominal aorta. Strut distortion also occurred during balloon retrieval in one patient, but no aneurysm or in-stent restenosis was observed at 1-year follow-up. The patients were followed for a median of 14 (4-25) months and none required redilation. CONCLUSIONS: Our initial results demonstrated that the BeGraft aortic stent effectively reduced the pressure gradient in selected native and recurrent cases. Despite advantages such as a smaller sheath and low profile, more experience and medium- to long-term results are needed.

Angioplasty of Native Coarctation in a Very Low Birth Weight, Donor of Twin-Twin Transfusion Infant.

We present a case of successful balloon angioplasty of native aortic coarctation in a preterm infant, a donor of twin-twin transfusion syndrome with low birth weight. Angioplasty was done at the age of 15 days and weight of 480 g, using umbilical artery approach. Follow-up at 4 months of age showed no recurrence of coarctation.

Effect of coarctation of aorta anatomy and balloon profile on the outcome of balloon angioplasty in infantile coarctation.

OBJECTIVE: Coarctation of the Aorta (CoA) is a relatively common cardiovascular disorder. The present study aimed to evaluate the effect of COA anatomy and high versus low-pressure balloons on the outcome of balloon angioplasty among neonates and infants. METHODS: In this retrospective study, the neonates and infants undergoing balloon angioplasty at Namazi hospital were enrolled. After balloon angioplasty, immediate data results were promptly recorded.Moreover, midterm echocardiographic information was collected via electronic cardiac records of pediatric wards and clinical and echocardiographic data at least 12 months after balloon angioplasty. Finally, data were analyzed using SPSS-20. RESULTS: In this study, 42 infants were included. The median age at the time of balloon angioplasty was 1.55 (range 0.1-12) months and 66.7% of the patients were male. The mean pressure gradient of coarctation was 38.49 ± 24.97 mmHg, which decreased to 7.61 ± 8.00 mmHg (P < 0.001). A high-pressure balloon was used in 27, and a low-pressure balloon was used in 15 patients. COA's pressure gradient changed 30.89 ± 18.06 in the high-pressure group and 24.53 ± 20.79 in the low-pressure balloon group (P = 0.282). In the high-pressure balloon group, 14.81% and in the low-pressure group, 33.33% had recoarctation and need second balloon angioplasty (p < 0.021). The infant with discrete coarctation had a higher decrease in gradient and lower recoarctation. CONCLUSION: Recoarctation rate was lower in the high-pressure balloon. The infant with discrete COA had a better response to the balloon with more decrease in gradient and lower recoarctation rate. Therefore, the stenotic segment anatomy needs to be considered in the selection of treatment methods.