Branch Pulmonary Artery Research Articles

Kidney and vascular involvement in Alagille syndrome.

Alagille syndrome (ALGS) is an autosomal dominant, multisystemic disease with a high interindividual variability. The two causative genes JAG1 and NOTCH2 are expressed during kidney development, can be reactivated during adulthood kidney disease, and Notch signalling is essential for vascular morphogenesis and remodelling in mice. Liver disease is the most frequent and severe involvement; neonatal cholestasis occurs in 85% of cases, pruritus in 74%, xanthomas in 24% of cases, and the cumulative incidences of portal hypertension and liver transplantation are 66% and 50% respectively at 18years of age. Stenosis/hypoplasia of the branch pulmonary arteries is the most frequent vascular abnormality reported in ALGS. Kidney involvement is present in 38% of patients, and can reveal the disease. Congenital anomalies of the kidney and urinary tract is reported in 22% of patients, hyperchloremic acidosis in 9%, and glomerulopathy and/or proteinuria in 6%. A decreased glomerular filtration rate is reported in 10% of patients and is more frequent after liver transplantation for ALGS than for biliary atresia. Kidney failure has been frequently reported in childhood and adulthood. Renal artery stenosis and mid aortic syndrome have also frequently been reported, often associated with hypertension and stenosis and/or aneurysm of other large arteries. ALGS patients require kidney assessment at diagnosis, long-term monitoring of kidney function and early detection of vascular complications, notably if they have undergone liver transplantation, to prevent progression of chronic kidney disease and vascular complications, which account for 15% of deaths at a median age of 2.2years in the most recent series.

Suppression of Aseptic Inflammation Reduces the Severity of Remodeling of the Pulmonary Artery Branches and Improves Progressing of Experimental Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism, characterized by increased pressure in the pulmonary artery and impaired lysis of thromboemboli. Previously, the presence of aseptic inflammation in CTEPH was identified in the wall of the pulmonary artery branches and perivascularly. However, the role of this inflammation in the CTEPH formation is unknown. The aim of the work was to study the effect of aseptic inflammation on the CTEPH formation and progression. The experiments were performed on 54 male rats. The CTEPH model was reproduced by repeated intravenous administration of partially biodegradable microspheres (MS). Immediately after the last administration of MS, all animals were divided into groups: control CTEPH (c.CTEPH) – saline solution was administered intramuscularly (i/m) for 6 weeks; low dose of prednisolone (LD) – prednisolone was administered i/m at a dose of 1.5 mg/kg; high dose (HD) – prednisolone was administered i/m at a dose of 6 mg/kg; healthy animals. After 6 weeks, the following was performed: treadmill test, TTE, cardiac catheterization with manometry, and histological examination of the lungs. In a separate series of experiments, the severity of inflammatory infiltration of the vascular wall and perivascular zone was assessed by immunohistochemical studies (IHC). In the LD group, there was the decreasing of hypertrophy index (HI) and the percentage of collagen fibers in the vascular wall compared to c.CTEPH. There was a significantly greater reduction in HI compared to HD. In the HD group, there was positive effect on the percentage of collagen fibers in the vascular wall, this parameter did not significantly differ from healthy animals. According to IHC data, prednisolone in low dose effectively suppressed inflammatory infiltration of the vascular wall and perivascular space. The results of the study revealed the ability of prednisolone, by suppressing aseptic inflammation, to reduce the severity of remodeling of the pulmonary artery branches.

Evaluation of an engineered vascular graft exhibiting somatic growth in lambs to model repair of absent pulmonary artery branch

BackgroundGrowth is the holy grail of tissue implants in pediatrics. No vascular graft currently in use for surgical repairs of congenital heart defects has somatic growth capacity.MethodsBiologically-engineered grafts (6 mm) grown from donor ovine fibroblasts in a sacrificial fibrin gel were implanted into the left pulmonary branch of 3-month old lambs for 3, 6, and 18 months. A control group of Propaten® PTFE grafts was implanted for 6 months.ResultsThe engineered grafts exhibit extensive site-appropriate recellularization after only 3 months and near-normal increase of diameter from the preimplant value of 6 mm to 12.9 mm and also a doubling of length from 6.0 mm to 13.0 mm at 6 months (n = 3) associated with apparent somatic graft growth (collagen content increase of 265% over 18-month, n = 2), along with excellent hemodynamics and no calcification, in contrast to the Propaten® grafts. The left-right flow distribution is nearly 50–50 for the engineered grafts at 6 months (n = 3) compared to about 20–80 for the Propaten® grafts (n = 3), which have less than one-half the diameter, a 6-fold higher pressure gradient, and stunted vascular development downstream of the graft. The engineered grafts exhibit a stable diameter over months 12–18 when the lambs become adult sheep (n = 2).ConclusionsThis study supports the use of these regenerative grafts with somatic growth capacity for clinical trial in patients born with a unilateral absent pulmonary artery branch, and it shows their potential for improving development of the downstream pulmonary vasculature.

Preoperative evaluation of the segmental artery of left upper lobe by thin-section CT and 3d-CTA.

Understanding pulmonary artery (PA) branches and their variations is crucial for successful lung resection. We aimed to evaluate the segmental PA branching pattern of the left upper lobe (LUL) using thin-section computed tomography (TSCT) images and 3D-CT angiography (3D-CTA). This study included 108 patients who underwent CTA and left upper lobectomy. The segmental PA branching pattern of the LUL was meticulously identified by two thoracic radiologists using 3D-CTA and TSCT images. The lingular artery branches from the left PA (LPA) were classified into mediastinal type (pars mediastinalis: PM), interlobar type (pars interlobaris: PI), and PI originating from the lower portion (PI'), specifically from A8. The intraoperative findings of the PA branches of the LUL were compared with the preoperatively obtained 3D-CTA and TSCT images in each patient's case. The median (range) number of LPA branches of the LUL was 4.36 (3-8). The most common number of A1 + 2 branches was two, seen in 34 cases (31.5%). One or more branches of A1 + 2c directly originating from the LPA were found in 63 cases (58.3%). The number of branches of A3 was single in 85 cases and the most frequent (78.7%). Instances where one or more branches of A3a directly originated from the LPA were found in seven cases (6.5%). A1 + 2 and A3 origins were separate and independent in 40 cases (37.0%). As the branching pattern of the lingular artery, PI/PI' was most frequent (61.1%). PI´ was observed in 26 cases (24.1%). Inter-observer agreement for A1 + 2, A1 + 2c, A3, A3a, and lingular artery branching patterns was moderate to substantial (κ = 0.53-0.72). Preoperative 3D-CTA and TSCT images identified 99.8% of LPA branches compared to intraoperative findings, except one. The segmental PA branching pattern of the LUL can be evaluated using TSCT and 3D-CTA images, providing precise preoperative information.

Operations for increasing the pulmonary blood flow in newborns with cyanotic congenital heart defects: results and features of outpatient postoperative monitoring

Background. In patients with cyanotic congenital heart defects, pulmonary blood flow is maintained by a functio­ning patent ductus arteriosus (PDA). Most patients with complex ductal-dependent cyanotic defects require intermediate staged treatment before radical correction of the defect. Timely and comprehensive outpatient monitoring by a pediatrician and pediatric cardiologist are important for patient survival following palliative treatment, along with determining optimal timing for consultations at specialized cardiac surgical centers. Objective: to present the outcomes of using two methods for increasing pulmonary blood flow (systemic-to-pulmonary artery shunt (SPAS) and PDA sten­ting), as well as the features of outpatient cardiological observation and treatment in these patients. Materials and methods. From 2000 to February 2024, 22 patients underwent SPAS, and 25 — PDA stenting at the State Institution “Scientific and Practical Medical Center of Pediatric Cardiology and Cardiac Surgery” of the Ministry of Health of Ukraine. Results. After interventions, the mean arterial oxygen saturation (SatO2) increased in both groups, significantly higher in the PDA stenting group (p < 0.05). The ave­rage length of stay in the intensive care unit in the SPAS group was 19.6 ± 11.1 (range: 5 to 91) days compared to 12.8 ± 6.3 (range: 4 to 37) days in those with PDA stenting (p = 0.05). The duration of artificial lung ventilation in the SPAS group was 290.3 ± 215.3 (range: 63 to 751) hours, and in the PDA stenting group, it was shorter, 151.8 ± 75.5 (range: 39 to 549) hours (p < 0.05). Early (30-day) postoperative mortality in the SPAS group was 13.6 % (3/22 patients), with a late mortality of 18 % (4/22). In contrast, there was not early (30-day) postoperative mortality in the PDA stenting group, and late mortality was 8 % (2/25). Before the subsequent stage of surgical correction, sufficient growth of pulmonary artery branches was noted (Nakata index increased from 156.9 ± 33.3 mm2/m2 to 277.0 ± 35.9 mm2/m2 in the SPAS group and from 142.7 ± 55.2 mm2/m2 to 289.1 ± 149.2 mm2/m2 in the PDA stenting group), and the left ventricular end-diastolic index has increased (from 51.2 ± 32.4 mm2/m2 to 67.5 ± 15.5 mm2/m2 in the SPAS group and from 50.8 ± 24.9 mm2/m2 to 56.7 ± 28.5 mm2/m2 in the PDA stenting group). Thirteen patients in the SPAS group underwent the next stage of surgical correction (Glenn shunt or total repair of the congenital heart defect), while in the PDA stenting group — 17 patients. Conclusions. For cyanotic congenital heart defects, which have ductus-dependent pulmonary blood flow, both described methods are quite effective.

Pharmacomechanical thrombectomy in management of pulmonary embolism

BackgroundAcute pulmonary embolism is recorded as one of the most common and critical medical conditions, resulting in a notable mortality rate requiring a multidisciplinary management. In this series, we address the management of massive and sub-massive pulmonary embolism utilizing catheter-based intervention, in particular the conjunction of mechanical thrombus disruption and aspiration with pharmacological thrombolysis (pharmacomechanical thrombectomy).Results37 patients were diagnosed with massive and sub-massive pulmonary embolism based on the clinical and radiological findings. Pre-procedural vital parameters were obtained, including oxygen saturation, pulse rate, and blood pressure. Under continuous monitoring and conscious sedation, a pulmonary angiography via right common femoral vein access was performed for pre-procedural assessment. Pharmacomechanical thrombectomy was performed using the AngioJet Ultra System (Boston Scientific). Using the power pulse option, a fibrinolytic agent was infused into the thrombus. After 5–10 min, mechanical thrombectomy is performed with a maximum 3 passes through the thrombus. This technique is performed in the main pulmonary artery and lower branch. The procedure is repeated on the other side. The maximum duration of thrombectomy is 2–3 min on each side. Procedure success was based on improvement of vital signs and not related to post-procedure angiographic findings. All patients showed immediate improvement of vital signs (blood pressure, 02 saturation, and pulse rate) with progressive improvement over the following days. There were no procedure-related complications.ConclusionPharmacomechanical thrombectomy is a safe and effective technique in the treatment of massive and submassive pulmonary embolism. It can be a first-line treatment even in patients without absolute contraindication to systemic thrombolysis.

The effects of percutaneous branch pulmonary artery interventions in biventricular congenital heart disease: study protocol for a randomized controlled Dutch multicenter interventional trial

BackgroundBranch pulmonary artery (PA) stenosis is one of the most common indications for percutaneous interventions in patients with transposition of the great arteries (TGA), tetralogy of Fallot (ToF), and truncus arteriosus (TA). However, the effects of percutaneous branch PA interventions on exercise capacity remains largely unknown. In addition, there is no consensus about the optimal timing of the intervention for asymptomatic patients according to international guidelines. This trial aims to identify the effects of percutaneous interventions for branch PA stenosis on exercise capacity in patients with TGA, ToF, and TA. In addition, it aims to assess the effects on RV function and to define early markers for RV adaptation and RV dysfunction to improve timing of these interventions.MethodsThis is a randomized multicenter interventional trial. TGA, ToF, and TA patients ≥ 8 years with a class IIa indication for percutaneous branch PA intervention according to international guidelines are eligible to participate. Patients will be randomized into the intervention group or the control group (conservative management for 6 months). All patients will undergo transthoracic echocardiography, cardiac magnetic resonance (CMR) imaging, and cardiopulmonary exercise testing at baseline, 6 months, and 2–4 years follow-up. Quality of life (QoL) questionnaires will be obtained at baseline, 2 weeks post intervention or a similar range for the control group, and 6 months follow-up. The primary outcome is exercise capacity expressed as maximum oxygen uptake (peak VO2 as percentage of predicted). A total of 56 patients (intervention group n = 28, control group n = 28) is required to demonstrate a 14% increase in maximum oxygen uptake (peak VO2 as percentage of predicted) in the interventional group compared to the control group (power 80%, overall type 1 error controlled at 5%). Secondary outcomes include various parameters for RV systolic function, RV functionality, RV remodeling, procedural success, complications, lung perfusion, and QoL.DiscussionThis trial will investigate the effects of percutaneous branch PA interventions on exercise capacity in patients with TGA, ToF, and TA and will identify early markers for RV adaptation and RV dysfunction to improve timing of the interventions.Trial registrationClinicalTrials.gov NCT05809310. Registered on March 15, 2023.

Pulmonary Arteriovenous Malformation in a Young Man

ABSTRACT A young man was diagnosed to have pulmonary arteriovenous malformation (PAVM) when he was admitted with COVID infection. He had no respiratory distress but his saturation at room air was 90%. Chest X-ray revealed non-homogenous opacity in left lower lobe (LLL). The heart was structurally normal on 2D echocardiography; the saline bubble study was however positive for extra-cardiac shunt. Computed tomography pulmonary angiography (CTPA) revealed PAVM with well-defined lobulated sac measuring 2.9 × 1.9 × 2.5 cm in lateral basal segment of LLL with segmental branch of left pulmonary artery as feeder vessel and it drained into left pulmonary vein. The patient was managed with percutaneous intervention using Amplatzer Vascular Plug II to occlude the feeder vessel. Post-intervention, patient’s saturation improved to 98% at room air and saline bubble study was negative for extra-cardiac shunt. CTPA at 06 weeks revealed complete thrombosis of the sac.

Стентування відкритої протоки як перший етап хірургічного лікування тетради Фалло з незливними гілками легеневої артерії

The aim – to show the results of patent ductus arteriosus (PDA) stenting as the first stage of palliative treatment in patients with tetralogy of Fallot with non-confluent branches of the pulmonary artery.Materials and methods. This retrospective, single-center study included 10 consecutive patients who underwent PDA stenting since 2013 to 2023. The average age at the time of surgery was 96.00 ± 82.92 days (range 4 to 411 days), the average weight was 5.10 ± 1.78 kg (range 2.8 to 11 kg). The arterial oxygen saturation level (SaO2) before the intervention was 72.0 ± 6.7 % (range 60 to 87 %).Results. There were no intraoperative complications in all patients of this group. The length of the stay in the intensive care unit after surgery was 5.00 ± 1.36 days. SaO2 level increased to 89.00 ± 4.08 %. The duration of artificial lung ventilation after surgery was 36.5 ± 18.00 hours, and the duration of inotropic support was 81.1 ± 33.8 hours. In the long term, all patients showed significant growth of the native pulmonary artery branches. All patients of this group achieved total repair of this pathology after 192.00 ± 60.28 days, on average. At the moment of total repair, the size of the left branch of the pulmonary artery increased from 3.9 ± 0.8 mm to 7.8 ± 1.6 mm, and the right branch increased from 5.8 ± 1.2 mm to 7.70 ± 1.04 mm; the Nakata index increased from 133.0 ± 29.1 mm2/m2 to 241.2 ± 97.8 mm2/m2; end-diastolic index of the left ventricle increased from 31.4 ± 10.8 ml/m2 to 43.1 ± 11.4 ml/m2.Conclusion. PDA stenting in patients with tetralogy of Fallot combined with non-confluent branches of the pulmonary artery is an effective palliative procedure. This method allows to postpone the total repair to an older age, ensures sufficient and symmetric growth of the branches of pulmonary artery, and helps to avoid risks associated with repeat open heart surgeries.

Retrograde Flow to Aortic Root Predicts Inferior Cardiopulmonary Performance and Restrictive Lung Physiology in Fontan Circulation

BackgroundCardiac output in Fontan circulation depends on systemic venous pressure, pulmonary vascular resistance, and ventricular function. Because myocardial function is dependent on coronary perfusion, we studied whether retrograde flow to aortic root in the mitral/aortic atresia subgroup of hypoplastic left heart syndrome (HLHS) affects cardiopulmonary performance. MethodsWe studied 26 stable Fontan patients (14.4 ± 2.4 years) with right (RV, n = 17) and left (LV, n = 9) systemic ventricle morphology. All RV patients had HLHS and were subdivided according to postnatal flow to the hypoplastic ascending aorta being antegrade (HLHS-A) or retrograde (HLHS-R) due to valve atresia. Physical activity was assessed by questionnaire (LASERI, a questionnaire for Finnish children regarding physical activity), cardiopulmonary exercise test (1-minute ramp protocol), body composition (Biacorpus RX 4000), and muscle fitness (EUROFIT). These data were correlated with the postnatal aorta size and current branch pulmonary artery size index (McGoon index). ResultsPatients with HLHS-R seldom self-reported engagement in vigorous physical activity and had significantly lower cardiopulmonary performance (peak oxygen uptake [VO2peak]) than patients with LV morphology (P = 0.037), but not compared with patients with HLHS-A. Branch pulmonary artery size did not correlate with VO2peak. Patients with HLHS-R had most severe lung restrictions (forced vital capacity z-score –3 ± 0.9, P = 0.0073; forced expiratory volume in 1 second z-score –3.3 ± 1.1, P = 0.001). ConclusionsYoung Fontan patients with LV had better cardiopulmonary performance than patients with HLHS. Patients with HLHS-R were the least active and had the lowest VO2peak and most restrictive lungs. It is important to recognize postnatally single ventricle patients at high risk for inactivity to promote an active and healthy lifestyle.

CT Imaging Findings of Pulmonary Artery Stenosis: A Pictorial Review.

Pulmonary artery stenosis represents a group of disorders involving main, branch or peripheral pulmonary arteries with pain, dyspnea, hemoptysis or even no symptoms. Early diagnosis and timely intervention are crucial for reducing mortality, but timely diagnosis is challenging due to the non-specific symptoms. Computed tomography pulmonary angiography (CTPA) is useful in the diagnosis because it can provide more details about abnormal changes in the lumen, vessel wall and adjacent mediastinal structures. Congenital and acquired pulmonary artery anomalies have some characteristics on CTPA, which can be useful for differential diagnosis. Awareness of these conditions is important for radiologists. This pictorial review provides an overview of CTPA imaging features of pulmonary artery stenosis.

Computational Study on the Effects of Valve Orientation on the Hemodynamics and Leaflet Dynamics of Bioprosthetic Pulmonary Valves.

Pulmonary valves do not display a fibrous annulus as do other valves in the heart; thus, pulmonary valves can be implanted at multiple orientations and locations within the right ventricular outflow tract (RVOT). This gives surgeons more freedom when implanting the valve but it also results in uncertainties regarding placement, particularly with respect to valve orientation. We investigate the pulmonary artery hemodynamics and valve leaflet dynamics of pulmonary valve replacements (PVRs) with various orientations via fluid-structure interaction (FSI) models. A canonical model of the branching pulmonary artery is coupled with a dynamic model of a pulmonary valve, and from this we quantify the effect of valve implant orientation on the postvalvular hemodynamics and leaflet dynamics. Metrics such as turbulent kinetic energy (TKE), branch pulmonary artery flow distributions, projected valve opening area (PVOA), and pressure differentials across the valve leaflets are analyzed. Our results indicate that off-axis orientation results in higher pressure forces and flow and energy asymmetry, which potentially have implications for long-term durability of implanted bioprosthetic valves.

Safety and effectiveness of using Disposable Ultrasonic shears to coagulate 5–7 mm blood vessels: protocol for a prospective, multicenter, randomized, parallel controlled, non-inferiority clinical trial

BackgroundThe ultrasonic scalpel is widely used during surgery. It is safe and effective to close the pulmonary artery branch vessels of 7 mm or below with an ultrasonic energy device as reported. However, there have been no multicenter randomized clinical trial to assess the safety and effectiveness of using ultrasonic scalpel to coagulate 5–7 mm blood vessels in thoracic surgery.MethodsThis is a prospective, multicenter, randomized, parallel controlled, non-inferiority clinical trial. A total of 144 eligible patients planning to undergo lung or esophageal surgery will be randomly allocated to the experimental group and the control group. The investigational product (Disposable Ultrasonic Shears manufactured by Reach Surgical, Inc.) and the control product (Harmonic Ace + 7, 5 mm Diameter Shears with Advanced Hemostasis) will be used in each group. The primary endpoint is the success rate of coagulating target blood vessels during surgery. Secondary endpoints include postoperative rebleeding, intraoperative bleeding volume, drainage volume, surgical duration, etc. Postoperative follow-up before and after discharge will be performed.DiscussionThis clinical trial aims to evaluate the safety and effectiveness of using the investigational product (Disposable Ultrasonic Shears manufactured by Reach Surgical, Inc.) and that of the control product (Harmonic Ace + 7, 5 mm Diameter Shears with Advanced Hemostasis) to coagulate 5–7 mm blood vessels in thoracic surgery.Trial registrationClinicalTrials.gov: NCT06002737. The trial was prospectively registered on 16 August 2023, https://www.clinicaltrials.gov/study/NCT06002737.

PATENT DUCTUS ARTERIOSUS STENTING IN NEWBORNS AND INFANTS WITH CYANOTIC CONGENITAL HEART DEFECTS: EXPERIENCE AND OUTCOMES

Background. Among all congenital heart defects (CHD), one-third are cyanotic defects with duct-dependent pulmonary blood flow. Palliative interventions are necessary for children with complex cyanotic CHD, premature infants, and children with complex comorbidities. Ensuring a reliable source of pulmonary blood flow adequate for arterial oxygenation and native pulmonary artery branches growth are the primary goals of staged interventions. The classic approach to providing adequate pulmonary blood flow before total repair is systemic-to-pulmonary artery shunting. Currently, there is a growing trend towards the use of an alternative method - transcatheter patent ductus arteriosus (PDA) stenting. Aim: Demonstrate the experience of PDA stenting, immediate and long-term outcomes in newborns and infants. Materials and Methods. From 2009 to April 2024, PDA stenting was performed in 66 patients at the UCC. Among the all cohort, 49 (74.2%) were newborns (Group I), and 17 (25.7%) were infants (Group II). The average age was: Group I - 7.4±5.9 days (range 1 to 28 days), and Group II - 105.2±54.78 days (range 42 to 280 days). The average weight was: Group I - 3.0±0.49 kg (range 1.9 to 4.4 kg), and Group II - 5.2±1.4 kg (range 2.7 to 9 kg). This study analyzed pre-, intra-, and postoperative parameters including age, weight, presence of concomitant/genetic pathology, saturation level, pulmonary artery branches diameter, Nakata index, PDAs size, origin and type, duration of staying in the intensive care unit, duration of artificial lung ventilation and inotropic support, early and late postoperative complications, 30-day and inter-stage mortality rates in the two patient groups. Results. All cases of transcatheter stent implantation were performed by transfemoral access. The average ICU stay duration for Group I was 13.8±9.52 days (range 2 to 119 days), and for Group II was 6.4±3.64 days (range 2 to 20 days). The duration of artificial lung ventilation in Group I was 185.8±165.28 hours (range 2 to 2066 hours), and in Group II was 67.5±46.15 hours (range 4 to 248 hours). Inotropic support - on average for Group I - 151.6±81.06 hours (range 0 to 720 hours), and for Group II - 82.4±47 hours (range 0 to 211 hours). Saturation increased from an average of 65.7±11.03% to 85.2±5.7% in Group I (p<0.05), and from 66.8±10.9% to 87.4±4.24% in Group II (p<0.05). Early postoperative complications were observed in 34.6% (17/49) of Group I patients and 23.5% (4/17) of Group II patients. The early 30-day mortality rate was 8.1% (4/49) for Group I. The next stage of surgical treatment wasperformed on average after 12.9±10.2 months by 51% (25/49) of Group I patients and by 88% (15/17) of Group II patients, on average after 14.98±12 months post-stenting. The interstage (late) mortality rate was 6.1% (3/49) for Group I and 5.8% (1/17) for Group II. Conclusion. Considering the outcomes after PDA stenting in our cohort of patients, this procedure appears to be an effective operation for providing a reliable source of pulmonary blood flow in various anatomical variants of cyanotic CHD in both newborns and infants.

Tactics of Surgical Repair of Single Ventricle with Excessive Pulmonary Blood Flow and Obstruction of the Distal Arch of the Aorta in Newborns

Pulmonary artery stenosis with distal aortic arch reconstruction and coarctation of the aorta in newborns is an effective palliative procedure for single ventricle and high pulmonary hypertension on the way to total cavo-pulmonary anastomosis. The aim. To present a case of complex correction of a single ventricle of the heart with tricuspid valve atresia and high pulmonary hypertension, obstruction of the distal aortic arch and coarctation of the aorta, and a final effective Fontan procedure with a good long-term outcome. Case report. On December 15, 2011, a 9-day-old newborn patient M. was admitted for treatment at the Department of Cardiovascular Surgery of Odesa Regional Children’s Clinical Hospital with a diagnosis of: a single ventricle of the heart, transposition of the great arteries, tricuspid atresia, distal arch hypoplasia, coarctation of the aorta, patent ductus arteriosus, high pulmonary hypertension. The first stage of surgical treatment was carried out on December 19, 2011: main pulmonary artery banding, reconstruction of the distal arch of the aorta through modified Amato technique, closure of the patent ductus arteriosus, resection of the coarctation of the aorta and extended end-to-end aortoplasty. At the age of 1 year (December 27, 2012), the second stage was performed: bidirectional Glenn procedure with plastic surgery of bifurcation and right pulmonary artery branch. The third stage was performed at the age of 3 years 11 months (November 19, 2015): Fontan procedure with extracardiac conduit. Conclusions. Early elimination of pulmonary hypertension by pulmonary artery banding ensures the preservation of the pulmonary vascular bed with low resistance, which is a crucial condition for the effective final hemodynamic correction of the single ventricle of the heart – the Fontan procedure. In case of hypoplasia of the distal aortic arch with coarctation in newborns, one of the alternative approaches is the use of modified Amato technique avoiding artificial circulation.

Absent Pulmonary Valve Syndrome with Non-Confluent Branches: Case Report and Literature Review

Absent pulmonary valve syndrome (APVS) is a rare form of congenital heart defect. Among the various variations of this rare anomaly are non-confluent branches of the pulmonary artery (PA) with anomalous origin of one of the PA branches from the patent ductus arteriosus (PDA). Constriction of the PDA in early neonatal age may result in cessation of blood supply to one of the pulmonary artery branches, leading to exclusion of one lung from circulation and gas exchange processes. PDA stenting creates a reliable source of blood flow, and as a first step, an endovascular approach was chosen for treatment. Case report. A newborn (3 kg) with a prenatal diagnosis of APVS and a non-confluent left PA branch filling through PDA was chosen for PDA stenting. Considering the complex anatomy of the heart defect, it was decided to perform PDA stenting to delay total repair until the patient reaches optimal age and weight. Our patient successfully underwent PDA stenting as the first stage, which provided conditions for blood supply to one lung. The second stage involved total repair with good long-term results. Conclusion. APVS treatment may have several options of surgical correction, differing from each other depending on the anatomical features of the defect and the patient’s age. Palliative treatment options help the child transition from the neonatal period and gain optimal weight for total repair.. Analyzing the data from the presented literature, it is noted that prenatal diagnosis is an important stage in determining the urgency and choice of treatment tactics for this defect.

Palliative Right Ventricular Outflow Tract Stenting in Tetralogy of Fallot with Severe Cyanosis: Experience from a Single Center in Southern India

Background: This study conducted at an Indian tertiary care hospital aimed to characterize the clinical profile of tetralogy of Fallot (ToF) patients undergoing right ventricular outflow tract (RVOT) stenting and assess their immediate postprocedural outcomes until discharge. Methods: In this retrospective analysis, the clinical and procedural outcomes were analyzed for 20 patients who underwent palliative stenting in the RVOT for severe cyanosis associated with ToF during 2021–2023. Results: The median age for the patients was 2.3 years (range 3 months–21 years), two being adults (>18 years). Fourteen (70%) patients underwent elective RVOT stent and 30% underwent emergency stenting. The most common indication of RVOT stenting was hypoplastic branch pulmonary arteries (PAs) (n = 11, 78.6%) and cyanotic spell (n = 4, 66.7%) for elective and emergency surgeries, respectively. The average improvement in SpO2 was 27.2% (95% confidence interval 23.4–31.0, P < 0.001). Six (30%) patients had complications including reperfusion injury of lungs (n = 2, 33.4%) sepsis (n = 1, 16.7%), stent embolization (n = 1, 16.7%), re-stenting (n = 1, 16.7%), transient complete heart block (n = 1, 16.7%) during the procedure, and. One child went through RVOT stent postoperative due to severe residual stenosis and child developed severe tricuspid regurgitation and succumbed to death due to culture positive sepsis with multi-organ dysfunction. Rest (n = 19, 95%) were discharged with an average 6.7 days of hospital stay. Conclusion: ROVT stenting is a safe and effective palliative management in ToF enhancing oxygen saturation prior to definitive surgical repair.

Diagnostic and prognostic values of serum Selenoprotein P and soluble St2 levels in pulmonary embolism

AimPulmonary Thromboembolism (PTE) occurs as a result of occlusion of one or more of the pulmonary artery branches by thrombus and is an important cause of right heart failure and pulmonary hypertension. Selenoprotein P (SePP) and soluble suppression of tumorigenicity 2 protein (sST2) are two new biomarkers that have previously been the subject of various studies in heart failure. The aim of this study was to determine the diagnostic and prognostic potential of SePP and soluble sST2 levels in patients with acute PTE. Materials and MethodsThe study included 135 patients diagnosed with acute non-massive PTE and 43 healthy volunteers. Clinical, laboratory, and radiological patient data were recorded. SePP and sST2 levels were measured in the patient and control groups. Patients were followed at 1, 3, and 6 months of treatment via the death notification system and telemedicine. ResultsSePP and sST2 levels were significantly lower in the patient group compared with the control group (SePP: 17.65 ng/ml vs. 43.06 ng/ml and sST2: 10.86 ng/ml vs. 16.20 ng/ml, both p < 0.001). No correlation was found at 1, 3, and 6 months of follow-up with prognosis and mortality. ConclusionSePP and sST2 values were significantly lower in patients with acute PTE compared with the control group. Low levels of these biomarkers may be diagnostically valuable.

Bronchial artery embolization using small particles is safe and effective: a single center 12-year experience

Abstract Background Bronchial artery embolization (BAE) using particles is an established treatment for hemoptysis. The use of polyvinyl alcohol (PVA) with a particle size of 300 µm or larger is thought to reduce the risk of non-target embolization but may result in more proximal vessel occlusion than is ideal, resulting in a high rate of early recurrent hemorrhage. Objective This study evaluates the safety and efficacy of BAE using PVA particles with a size of less than 300 µm. Methods All patients who underwent BAE between 2010 and 2022 at a tertiary center were included. Demographic data, etiology and volume of hemoptysis, technical and clinical success, procedure-related complications, and follow-up information were collected from patients’ electronic records. 150–250 µm PVA particles were used to commence embolization in all patients with the subsequent use of larger-sized particles in some individuals. The Kaplan–Meier method was used to estimate recurrence and survival rates. Results One hundred forty-four patients underwent 189 embolization procedures between 2010 and 2022 and were followed up for a median of 35 months [IQR 19–89]. 150 µm to 250 µm PVA particles were used as the sole embolic agent in 137 cases. Hemoptysis recurred within 30 days in 7%. The median time to repeat intervention was 144 days [IQR 42–441]. Seventeen out of 144 patients had a pulmonary artery branch pseudoaneurysm. The rate of major complications was 1% with no instances of stroke or spinal artery ischemia. Thirty-day mortality was 2% (4/189). Conclusion BAE using 150–250 µm PVA particles is safe and effective with few complications and low rates of early hemoptysis recurrence. Clinical relevance statement BAE using small particles is likely to improve outcomes, particularly the rate of early recurrence, in patients with hemoptysis, without an increase in procedural complications. Key Points BAE is a safe and effective treatment for patients with hemoptysis. Using small PVA particles in BAE has few complications and low rates of early recurrence. Pulmonary artery pseudoaneurysms should be actively sought in those with hemoptysis undergoing BAE.

Pulmonary Embolism of the Small Branches of the Pulmonary Artery as a Result of Combat Trauma: Review of the Literature and Analysis of a Clinical Case

Pulmonary embolism (PE) is recognized by WHO as one of the most common cardiovascular diseases. Even in peacetime, PE is the third leading cause of sudden death after coronary heart disease and stroke. Today, as a result of the war, there have been significant corrections in the understanding of etiology, pathogenesis, features of the clinical course and treatment of internal pathology in the wounded persons. It was established that an important feature of modern combat activity is a constant increase in the frequency of multiple and combined wounds, while combat injuries of the chest make up to 15% of the structure of sanitary losses, and closed chest injuries under combat conditions can make up to 5%. Diseases of the cardiovascular and respiratory system are observed both early and late after closed chest trauma. The early ones are represented by functional disorders of the blood circulation and breathing system and are the consequences of damage to the chest organs, and the late ones occur in the case of joining a wound infection and are characterized by inflammatory processes in the lungs, pleura, heart and blood vessels, include pneumonia, pleurisy, acute purulent-destructive diseases lungs, thromboembolic complications. The article analyzes the course and possibilities of diagnosis and treatment of PE using the example of a wounded military man. A clinical case is presented which describes the successful diagnosis and treatment of a wounded person with PE of small branches of the pulmonary artery with the development of infarct-pneumonia S8, S9 of the right lung, nosocomial pneumonia in the right lung, which was complicated by the destruction of lung tissue, with the formation of an abscess-hematoma, acute interstitial nephritis, complicated by acute kidney damage of the III degree. This therapeutic pathology arose after a gunshot shrapnel penetrating wound of the chest on the right side with damage to the right subclavian vein, migration and embolization by a metal fragment of the right pulmonary artery, a gunshot shrapnel blind wound of the upper third of the right forearm with a gunshot fracture of the ulna and the presence of a foreign body (debris) in the muscle what tissues of the forearm. During the operation a complication arose in the form of migration of a foreign body from the branches of the right pulmonary artery to the branches of the left pulmonary artery. The result of the patient’s treatment was positive dynamics of the pathological process. The serviceman was discharged from the hospital in satisfactory condition and returned to the Armed Forces of Ukraine after a medical treatment. PE of small branches of the pulmonary artery is a severe complication after gunshot shrapnel injury to the chest organs, which can occur without concomitant deep vein thrombosis of the lower extremities, most likely as a result of the development of shock and the migration of fragments along the branches of the pulmonary artery. Complex treatment with the use of surgical methods and adequate antibacterial therapy is the main method of treatment of chronic destruction of lung tissue with abscessation. Each patient needs a complex of imaging methods for dynamic examination and monitoring of the state of other organs and systems, in particular, kidney function.