Lung ultrasound score in the decision of patent ductus arteriosus closure in neonates.

PURPOSE: We aimed to investigate the role of lung ultrasound (LUS) score in the closure of hemodynamically insignificant patent ductus arteriosus (PDA) and the clinical findings of the patients before and after closure. METHODS: The study groups (107 preterm neonates under 34 gestational weeks) were classified as hemodynamically significant PDA (group 1), hemodynamically insignificant PDA with closure therapy (group 2), hemodynamically insignificant PDA without closure therapy (group 3), and no PDA group (group 4) based on the echocardiography. 6- and 10-region LUS scores were compared for each group. RESULTS: There was a significant difference between groups 1 and 3 on first, third, and seventh days. In contrast, groups 1 and 2 had similar LUS scores on the first, third, and seventh days. There was a negative correlation between LUS scores on the first and third days and gestational age, birth weight, the first- and fifth-minute APGAR scores, and there was a positive correlation between aortic root to left atrium ratio, and PDA diameter/weight ratio. CONCLUSION: We observed that LUS scores in patients with hemodynamically insignificant PDA treated with closure therapy were similar to in patients with hemodynamically significant PDA. Thus, LUS score can have role in PDA closure in preterm neonates. However, more comprehensive studies are needed.

Expanding the phenotypic landscape of Gaucher disease type 3c with a novel entity - Transient neonatal cholestasis.

Gaucher disease (GD) is the most frequent lysosomal storage disorder due to biallelic pathogenic variants in GBA gene. Only homozygous D409H variant has been associated with the cardiovascular phenotype which is also known as Gaucher disease type 3c. In this descriptive study, we presented phenotypic heterogeneity and a novel clinical finding among 13 patients with GD type 3c. Patients presented with varying degrees of cardiac valve and/or aortic calcifications (84,6%) and corneal opacities (76,9%) in addition to visceral (100%), hematological (92,3%), neurological (92,3%), and skeletal (30%) manifestations. Also, cervical dystonia (38,4%) and psychiatric disorders (46,1%) were not infrequent entities with respect to neurological involvement in GD type 3c. In this report, we highlight transient neonatal cholestasis (38,4%) as a novel finding in GD type 3c. Neonatal cholestasis is a finding associated with Gaucher type 2, but transient neonatal cholestasis has not been reported in GD patients, so far. The clinical features of GD type 3c are highly heterogeneous, from disease severity or age of onset to disease progression. Also, we concluded that phenotypic spectrum may be associated with age at onset of clinical symptoms. As, patients presenting in infancy or childhood had mainly visceral and hematological involvement and patients presenting in adolescence and adulthood had mainly cardiac, neurological involvement, and psychiatric behavioral disorders. Identifying the heterogeneous clinical course of these patients in this fatal disease, may lead a sufficient understanding of the pathophysiology which will enable targeted therapeutic interventions.

Anomalous origin of coronary arteries from the pulmonary artery: A single-center experience.

Background: The aim of the study was to evaluate the clinical and diagnostic findings, treatment, and follow-up of cases of anomalous origin of coronary arteries from the pulmonary artery. Methods: Between January 1998 and June 2021, a total of 14 patients (5 males, 9 females; median age: 15 months; range, 3 to 156 months) diagnosed with anomalous origin of coronary arteries from the pulmonary artery were retrospectively analyzed. Demographic and clinical data of the patients, electrocardiographic, echocardiographic, angiographic, surgical, and follow-up findings were evaluated. Results: The most common symptoms were respiratory distress (n=6) and murmur (n=3). With the exception of three cases, all other patients were diagnosed by echocardiography in the first examination. Severe mitral valve insufficiency was detected in four patients and four other patients had moderate mitral insufficiency on echocardiography. Ejection fraction values ranged between 38 and 79%. Eleven patients underwent direct implantation of the coronary artery into the aorta, and three underwent a Takeuchi procedure. Mortality occurred in only one case. After surgery, mitral insufficiency and ejection fraction values improved. Median follow-up was 62 (range, 5 to 170) months and all patients were asymptomatic, except one who required redo surgery. Conclusion: Anomalous origin of the coronary arteries from the pulmonary artery is an uncommon congenital anomaly. Echocardiography is the main diagnostic tool. If all echocardiographic findings of the coronary anomaly are not investigated thoroughly, the diagnosis may be overlooked.

Cardiac effects of multisystem inflammatory syndrome in children: One-year follow-up.

AIM: Cardiovascular involvement is common among children with multisystem inflammatory syndrome (MIS-C) and can cause shock and death. In this study, we evaluated the early and long-term cardiac effects of MIS-C. METHODS: In this observational cohort study, we included all children treated for MIS-C from October 2020 to November 2021 in the Department of Paediatric Infectious Disease at Cukurova University School of Medicine Hospital. The patients underwent serial echocardiographical evaluation during hospitalisation and at 1, 3, 6 and 12 months after discharge. The patients were evaluated using Holter monitorisation between 4 and 6 months and using cardiac magnetic resonance imaging at 6 months and thereafter. RESULTS: Twenty-six patients diagnosed with MIS-C and with a median age of 84 months were included. Cardiac involvement was found in 19 (73.1%) patients. At initial echocardiographic evaluation, the mean ejection fraction value of the patients was 56.7% (range: 30-75). Coronary artery dilatation was detected in two (7.7%) patients, and mitral regurgitation persisted in only one patient by month 3. Treatment was started in two (7.7%) patients due to ventricular arrhythmia. Cardiac magnetic resonance imaging was performed in 13 (50%) patients at a median of 6 months (range: 5-9). The cardiac magnetic resonance imaging findings were consistent with possible interstitial fibrosis in two (7.7%) patients. CONCLUSION: Our results showed that cardiac involvement of patients improved rapidly with treatment, as indicated by previous studies. However, during the 1-year follow-up, frequent extraventricular systole was detected in two patients, one of whom initially did not show cardiac involvement. Moreover, possible interstitial fibrosis was detected in the cardiac magnetic resonance imaging (MRI) evaluation of two patients. In particular, we believe that these findings may be useful to evaluate critically ill paediatric patients and patients with severely low EF with cardiac MRI in their follow-up.

Infective endocarditis in childhood: a single-centre experience of 26 years.

The aim of this study was to present the clinical and microbiological characteristics of patients with infective endocarditis.A retrospective evaluation was made of patients diagnosed with infective endocarditis between 1995 and 2021. The clinical and laboratory characteristics of the patients were recorded together with conditions constituting a risk for the development of endocarditis, treatment, and surgical outcomes.Evaluation was made of 68 patients with a mean age of 7.3 years (3 months-17 years), diagnosed with infective endocarditis. An underlying cause of CHD was determined in 47 (69%) patients and rheumatic valve disease in 3 (4.4%). There was no structural heart disease in 18 (26%) patients of whom 13 of them had other risk factors. A causative organism was found in 41 (60%) cases, and the microorganism most often determined was viridans group streptococcus. No difference was determined between the patients diagnosed before and after 2007 in respect of the frequency of viridans streptococcus (p > 0.05). Septic emboli were seen in 18 (26%) patients, of which 17 required surgical treatments. In 5 of 11 (16.6%) patients with mortality, the agent was S. aureus. Of the total 28 (41%) patients were evaluated as hospital-acquired endocarditis. The most frequently determined agents in this group were staphylococcus epidermidis and S. aureus.Although CHDs continue to be the greatest risk factor for endocarditis, there is an increasing frequency of endocarditis in patients with no structural heart disease. Mortality rates are still high in infective endocarditis, especially in S. aureus endocarditis.

Ischemic-Modified Albumin in Neonates with Congenital Heart Defects.

OBJECTIVES: Ischemia-modified albumin (IMA) is a new biochemical marker of ischemia. We aimed to search blood IMA levels in neonates with congenital heart defects. STUDY DESIGN: During the study period, patients diagnosed with congenital heart disease and newborns with a diagnosis of hyperbilirubinemia as a control group were included in the study. IMA level was analyzed using the IMA absorbance unit (ABSU) method. RESULTS: In total, 57 newborns with congenital heart disease requiring cardiac operation for the study group and 38 newborns for the control group were included. There was no difference between the two groups in terms of gender, mode of delivery, and weeks of gestation. The average IMA values in the control group were 0.19 ± 0.09 ABSU. The prepostoperative mean IMA values of the patient group were 0.22 ± 0.07 and 0.23 ± 0.07 ABSU, respectively. Comparison of the postoperative IMA with the mean of the control group was statistically significant. Preoperative and postoperative IMA values of patients who have died due to primary heart disease and surgical complications were 0.21 ± 0.07 (0.08-0.32) ABSU and 0.25 ± 0.06 (0.12-0.36) ABSU, respectively. IMA levels were not statistically different between the two groups. CONCLUSION: Hypoxia and ischemia in congenital heart disease in the newborn period both preoperatively and postoperatively were important in prognosis. IMA was higher in the postoperative group. Many comprehensive studies are important in terms of preventing complications and decreasing mortality and morbidity by commenting on prognosis. KEY POINTS: · IMA is a new biochemical marker of ischemia.. · In the literature, there are no reports about the relation between congenital heart defects and IM.. · The exposure of CHD patients to hypoxia/asphyxia in the preintra and postoperative periods cause neurologic deficits.

Prenatal diagnosis of a right ventricular outpouching: a report of case.

We report a case of a right ventricular outpouching diagnosed during prenatal period. We defined it as an aneurysm because of its thin, hypokinetic wall, and wide neck connecting to the ventricle. Ventricular aneurysms, especially right ventricular aneurysms, are very rare cardiac malformations. Therefore, we describe a rare case of right ventricular aneurysm and the diagnostic features, differential diagnosis, and clinical features of ventricular aneurysms.

Clinical features and echocardiographic findings of isolated foramen ovale restriction in foetuses.

Isolated restrictive foramen ovale (rFO) without complex heart defects is a rare pathology. There may be difficulties in managing this situation, which can lead to right heart enlargement, tricuspid regurgitation and hydrops findings in the foetus. We conducted a retrospective analysis of 8451 foetuses. 7883 (93.2%) had a structurally normal heart or minor heart disease, 18 (0.22%) of which had a diagnosis of isolated rFO. Nine patients with neonatal echocardiographic examination were included in the study. In 8 (88.8%) patients, it was stated that a decision to give birth should be made at the time of presentation. Evaluating postpartum echocardiographic examinations, 7 (77.7%) patients had normal or minor defects. The decision of delivery made at the right time during follow-up is critical to determine the prognosis.IMPACT STATEMENTWhat is already known on this subject? The data about the prenatal diagnosis of isolated rFO is limited.What the results of this study add? We conducted a retrospective analysis of 8451 foetuses. 7883 (93.2%) foetuses had a structurally normal heart or minor heart disease, 18 (0.22%) of which had a diagnosis of rFO. Nine patients with foetal and postnatal follow-up and echocardiographic findings were examined in the study. The group's median gestational age at admission was 35.0 weeks (range: 27.0-39.0 weeks). The delivery decision was made in 8 (88.8%) patients at the time of admission. Evaluating postpartum echocardiographic examinations, 7 (77.7%) patients had normal or minor defects. Additionally, one patient was diagnosed with cardiomyopathy, and the other patient was diagnosed with functional pulmonary atresia. No death occurred in any foetus during follow-up.What the implications are of these findings for clinical practice and/or further research? Isolated rFO, a rare condition in the foetus, is generally well-tolerated in foetal life. However, the right heart enlargement, tricuspid regurgitation, or hydrops findings can be seen in patients. The decision of delivery made at the right time during follow-up is critical to determine the prognosis.

What are the non-cardiac prognostic factors affecting mortality in neonates with aortopulmonary shunt.

Background/aim: Systemic to pulmonary shunts (SPS) have proven to be highly effective for the palliation of neonates with cyanotic congenital heart disease. Mortality after SPS surgery in neonates has multifactorial basis. We aimed to investigate the clinical results of the SPS in relation to the underlying cardiac disease and to identify the risk factors contributing to an adverse outcome.Material and method: All neonates who underwent first shunt insertion for cyanotic congenital heart disease during the study period from 1 January 2014 to 31 December 2017 were included. A retrospective review of patient records was done. Patients were grouped into two different categories: survived with or without any reintervention and death before or after any reintervention till discharge.Result: During the study period, 47 patients underwent SPS shunt placement. Patients who survived with or without any reintervention were in Group 1 and patients who died before or after any reintervention till discharge were in Group 2. Preoperative epinephrine requirement and mechanical ventilation and postoperative erythrocyte transfusion need were statistically significant.Conclusion: Although primary cardiac pathology is the most important prognostic factor, some other preoperative and postoperative factors like preoperative epinephrine requirement, and postoperative erythrocyte transfusion might also affect the prognosis. As there are very few centers in the region that specialize in pediatric cardiac surgery, a multicenter approach will be helpful in reaching reliable conclusions.

A Case With Wiskott-Aldrich Syndrome and Ascending Aorta Aneurysm.

Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder that is characterized by a triad of microthrombocytopenia, severe immunodeficiency, and eczema. We report the case of a 7-year-old male patient with chronic thrombocytopenia that was diagnosed as WAS after dilatation of the ascending aorta was noticed. WAS is rare, and it is a disease that requires high suspicion for diagnosis. We recommend periodic echocardiography and magnetic resonance imaging examinations to evaluate aortic aneurysms in children with WAS and that surgical intervention should not be delayed when aneurysm is detected.

Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

[Low cardiac output syndrome score to evaluate postoperative cardiac surgery patients in a pediatric intensive care unit]. / Çocuk yogun bakim ünitesinde kardiyak cerrahi sonrasi hastalarin degerlendirilmesinde kullanilan düsük kardiyak debi sendromu skorlamasi.

OBJECTIVE: There is no clear consensus regarding the definition of low cardiac output syndrome (LCOS) or the follow-up of this patient group. Given this lack of a clinical definition, the aim of this study was to use a LCOS score (LCOSs) similar to the low cardiac output score previously presented in the literature and evaluate the relationship between a high LCOSs and poor clinical outcome. METHODS: A total of 54 patients were prospectively evaluated after cardiac surgery. The LCOSs was used to evaluate the deve-lopment of low cardiac output. Each parameter was scored as 1 point. The score was calculated every hour for 24 hours postoperatively and the highest score was recorded as the peak score (pLOCSs). The LOCSs at the time of admission to the pediatric intensive care unit, at the 4th, 8th, and 16th hour were recorded and a cumulative score (cLOCSs) score was calculated. RESULTS: The mean age of the patients was 49.40±53.15 months and 24.07% had LOCS. In the group with LCOS, the cLOCSs, vasoactive-inotropic score (VIS), lactate mean, aortic clamp time, and the total cardiopulmonary bypass time were significantly higher. In this study, a significant and positive correlation was found between the cLOCSs and pLOCSs and the length of hospital stay, length of stay in the pediatric intensive care unit, VIS, lactate mean, and aortic clamp duration. CONCLUSION: The objective of this study was to draw attention to the potential use of a common language in the care of critical pediatric patients undergoing cardiac surgery with a previously defined scoring method that includes parameters indicating poor perfusion in the patient.

Comparison of cardiac output and cardiac index values measured by critical care echocardiography with the values measured by pulse index continuous cardiac output (PiCCO) in the pediatric intensive care unit:a preliminary study.

BACKGROUND: Planning optimal fluid and inotrope-vasopressor-inodilator therapy is essential in critically ill children. Pulse index Contour Cardiac Output (PiCCO) monitoring is an invasive, hemodynamic monitor that provides parameter measurements such as cardiac output (CO), cardiac index (CI). Use of ultrasonography and critical care echocardiography by the pediatric intensivists has increased in recent years. In the hands of an experienced pediatric intensivist, critical echocardiography can accurately measure both CO and CI. Our objective in this study is to compare the CO and CI values measured by pediatric intensivist using critical care echocardiography to the values measured by PiCCO monitor in critically ill pediatric patients. METHODS: A prospective observational study from a tertiary university hospital PICU. A total of 15 patients who required advanced hemodynamic monitoring and applied PiCCO monitoring were included the study. The diagnosis of patients were septic shock, cardiogenic shock, acute respiratory distress syndrome, pulmonary edema. Forty nine echocardiographic measurements were performed and from 15 patients. All echocardiographic measurements were performed by a pediatric intensive care fellow experienced in cardiac ultrasound. The distance of left ventricle outflow tract (LVOT) in the parasternal long axis and LVOT-Velocity Time Integral (LVOT-VTI) measurement was performed in the apical five chamber image. Cardiac output_echocardiography (CO_echo) and CI_echocardiography (CI_echo) were calculated using these two measurements. PiCCO (PiCCO, Pulsion Medical Systems, Munich, Germany) monitoring was performed. Cardiac output (CO_picco) and CI (CI_picco) were simultaneously measured by PiCCO monitor and echocardiography. We performed a correlation analysis with this 49 echocardiographic measurements and PiCCO measurements. RESULTS: We detected a strong positive correlation between CO_echo and CO_picco measurements (p < 0.001, r = 0.985) and a strong positive correlation between CI_echo and CI_picco measurements (p < 0.001, r = 0.943). CONCLUSIONS: Our study results suggest that critical care echocardiography measurement of CO and CI performed by an experienced pediatric intensivist are comparable to PiCCO measurements. The critical care echocardiography measurement can be used to guide fluid and vasoactive-inotropic management of critically ill pediatric patients.

Comparison of Transcatheter Atrial Septal Defect Closure in Children, Adolescents and adults: Differences, Challenges and Short-, Mid- and Long-Term Results.

BACKGROUND AND OBJECTIVES: This study aims to compare the characteristics, effectiveness and results of transcatheter closure of atrial septal defect between children, adolescents, and adults. SUBJECTS AND METHODS: In this study, 683 patients who underwent atrial septal defect closure in the last 10 years were divided into three groups: children (age <12), adolescents (age 12 to 16), and adults (age >16) as group 1, group 2 and group 3, respectively. RESULTS: The average defect size and incidence of complex atrial septal defect were higher in group 3 (p=0.0001 and 0.03 respectively). While the average size of the devic was higher in adults (22.6±6.4 mm vs. 18.5±4.9 mm; p=0.0001), the ratio of the device size/total septum was higher in both children and adolescents (Group 1 and 2). In the child and adolescent groups and patients with only complex atrial septal defect, the use of techniques, other than standard deployment, was similar in all three groups (p=0.86 and 0.41, respectively). The ratio of the residual shunt was similar in all three groups. Major complications were seen in 5 cases (4 cases with migration, and 1 case with dislocation) in group 3 and 1 case (migration) in group 1. CONCLUSION: Depending on the complexity of the defect and age of the patient, transcatheter closure of atrial septal defect might have certain difficulties and complications. Patients must be evaluated in detail to avoid major complications and possible problems during the procedure.

Short-Term Outcomes of Patent Ductus Arteriosus Closure With New Occlutech® Duct Occluder: A Multicenter Study.

AIM: Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO. METHODS: We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA. RESULTS: The median patient age was 2.5 years (6 months-35 years), and median PDA diameter was 2.5 mm (1.2-11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24-48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months. CONCLUSION: Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325-331).

An Unusual Complication of the Ventricular Septal Defect Closure by Device: Late Right Aortic Cusp Perforation.

Aortic regurgitation (AR) is a rare complication of transcatheter closure of perimembranous ventricular septal defects (pmVSD). It results from iatrogenic pinching of the aortic valve by the VSD occluder or perforation by the catheter. It is usually detected during control echocardiography (ECHO). The current study reports the first case of a late AR, which resulted from late right coronary cusp perforation by the VSD occluder. The current manuscript discusses the possible causes of late cusp erosion due to occluder, advantages of early operation in such cases, and an alternative treatment method where the occluder removal is not possible at the operation.

Flow reduction of a neonatal stented arterial duct by covered coronary stent.

The final flow rate through a stented duct is variable and depends on stent diameter, ductal length, and ductal tissue protrusion after ductal constriction. Typically after duct stenting, there is initially mild overperfusion which may require some antifailure treatment. Severe heart failure is uncommon, but in some cases flow reduction is required. We present a case of overperfusion after arterial duct stent implantation which was successfully managed with implantation of covered stents.

Prenatal diagnosis of giant left ventricular diverticulum: case report.

The distinction between a ventricular aneurysm and diverticulum is difficult. The differences between these two are based on anatomical, histological, and functional criteria. We present a case of prenatal diagnosis of a giant left ventricular outpouching at 28 weeks gestation. During the postnatal period, the neonate underwent surgical correction for the outpouching. Histopathological examination reported the resected segment was a cardiac diverticulum. Our case indicates that differentiating between the two diagnoses during the intrauterine period is not always straightforward and possible. Histopathological examinations are sometimes necessary to make a precise differentiation. Treatment should be individualized depending on the clinical presentation.

Cardi-O-Fix duct occluder versus Amplatzer duct occluder for closure of patent ductus arteriosus.

BACKGROUND: We sought to investigate the safety, efficacy, and follow-up results of percutaneous patent ductus arteriosus (PDA) closure using the novel Cardi-O-Fix duct occluder (CDO), a device similar to but less expensive than the Amplatzer duct occluder (ADO). We also aimed to compare these two devices in terms of results. METHODS: Between March 2005 and May 2012, 167 patients diagnosed with moderate-to-large PDA underwent transcatheter closure. ADO was used in 56 (33.5%) patients with a mean age of 8.1 ± 11.9 years (3.6 months-56 years), whereas CDO was used in 111 (66.5%) patients with a mean age of 12.6 ± 14.6 years (4.8 months-63 years). RESULTS: The narrowest PDA diameter, the used device diameter, procedure time, fluoroscopy time, and residual shunt rates were similar between the two groups. Procedural success rate was 100% in both groups. Although the residual shunt rate was higher in the CDO group immediately after the procedure, the difference was not statistically significant (12.6 vs. 8.9%; P = 0.3). There was no statistically significant difference between groups at discharge and during follow-up. No deaths occurred in any of the groups, and there were no differences in complication rates during the short- and mid-term follow-up periods (CDO 7/111 vs. ADO 5/56; P = 0.5 π). CONCLUSION: The CDO can be used for PDA closure because of its safety, effectiveness, and simplicity in use. It is available in bigger sizes and can be used in patients with large defects. According to our short- and mid-term findings, the results it yields are similar to those of the ADO; thus, it may be the preferred choice owing to its low cost and large size variability.

Long-term follow-up results of lung perfusion studies after transcatheter closure of patent ductus arteriosus.

OBJECTIVE: This study presents the long-term follow-up of patients who developed left lung perfusion (LLP) abnormalities following patent ductus arteriosus (PDA) closure with various device types. DESIGN: The study includes 23 adult and pediatric patients who had undergone transcatheter PDA closure and were shown to have decreased LLP (<40%) by the first scintigraphy performed within the average follow-up period of 14.0 ± 8.12 months (2.0-30 months). For PDA closure, the Amplatzer duct occluder was used in 12 patients, and coils were used in 11. Within the average period of 58.91 ± 12.93 months (37-85 months) after transcatheter PDA closure, a second lung perfusion scintigraphy was performed. RESULTS: In 13 out of 23 patients (56.5%), LLP improved by the time of the second scintigraphy. Improved and unimproved patients did not differ with regard to age, weight, body surface area, PDA diameter, ampulla diameter, and PDA length at the time of PDA closure and the second scintigraphy. There was no significant difference with regard to the percent of improved patients between the different device types (P =.88). The left pulmonary artery indexes were also insignificantly different (P =.446). Patients with persistent LLP abnormality had significantly higher average Doppler velocity index [(LPA blood flow velocity--RPA blood flow velocity) / MPA blood flow velocity] × 100 (P =.007) and PDA diameter/length. If Doppler velocity index ≥50% is taken as the cutoff value, it is possible to predict persisting LLP abnormality with 80% sensitivity and 76% specificity. Left lung perfusion abnormality was found to persist in patients with PDA diameter/length ≥0.5 with 80% sensitivity and 92.3% specificity. CONCLUSIONS: The LLP abnormalities seen after PDA closure with various devices eventually improve to normal in the majority of patients during long-term follow-up. Patients whose PDA length is shorter than its diameter are at risk of developing LLP abnormalities that persist long-term.