Renal artery stenosis(RAS) is responsible for about 10% of secondary childhood hypertension that lead to end organ dysfunction and mortality. Angioplasty is gold intervention approach for stenosis treatment. However, there are rare studies in term of the pediatric RAS and angioplasty outcome in the children. In this Single-center, retrospective study, the medical records of pediatric RAS patients were analyzed who referred for Rajaei cardiovascular medical and research center of Tehran from 2005 to 2018. Of these, 13 patients underwent PTA (Percutaneous transluminal angioplasty) treatment. Demographic data, etiology, symptoms, presenting feature, stenosis, blood pressure, number of drugs, hematological and biochemical parameters, intervention type, treatment complication and echocardiography reports were reviewed. Statistical analysis was performed with SPSS Version 23 software package. Descriptive statistics, Chi square and Pearson correlation, parametric and non-parametric tests used for statistical analysis. Our results showed headache and cardiovascular damage was most common presenting feature. PTA intervention is effective in order of 50% of patients that reduced the blood pressure and improved medical therapy. The numbers of administrated antihypertensive drugs were reduced after PTA therapy. Restenosis and contrast induced-nephropathy emerge as major complications. Higher stenosis present but not lesion length had a negative relationship with treatment success and outcome. Age, ESR and serum potassium levels predicted the treatment outcome significantly. In conclusion, this study confirm the effectiveness of PTA in the treatment of pediatric RAS but need more technical and biomedical improvement to yield higher success and outcome.
Coronary cameral fistulas (CCFs) are rare congenital anomalies characterized by abnormal connections between a coronary artery and one of the cardiac chambers. These abnormal connections can lead to significant clinical implications, including heart failure and myocardial ischemia, necessitating timely diagnosis and intervention.
Percutaneous pulmonary valve implantation (PPVI) is a recognized alternative treatment to surgery for patients with dysfunctional right ventricular outflow tracts. Patient selection is essential to avoid serious complications from attempted treatment, such as rupture or dissection, especially of the calcified outflow tracts. We describe a case with an unexpected rupture of a calcified homograft valve and main pulmonary artery, which was treated successfully by emergency implantation of a self-expanding Venus P-Valve (Venus MedTech, Hangzhou, China) without the need for pre-stenting with a covered stent.
The duration of mechanical ventilation (MV) is one of the most important clinical factors which predict outcomes in pediatric cardiac surgery. The prolonged mechanical ventilation (PMV) following cardiac surgery is a multifactorial phenomenon and there are conflicts regarding its predictors in pediatric population between different centers.The current study aimed to describe PMV predictors in patients undergoing cardiac surgery for congenital heart disease in a tertiary center for pediatric cardiovascular diseases in Iran.From May to December 2014, all pediatric patients (less than a month - 15 years old) admitted to pediatric Intensive Care Unit (PICU) after congenital heart surgeries were consecutively included. The PMV was defined as mechanical ventilation duration more than 72 hours as medium PMV and more than seven days as extended PMV. The demographic data and variables probably related to PMV were recorded during the PICU stay.A total of 300 patients, 56.7% male, were enrolled in this study. Their mean age was 32 ± 40 months .The median duration (IQR) of MV was 18 hours (8.6 - 48 hours). The incidence of PMV more than 72 hours and seven days was 20% and 10.7%, respectively. Younger age, lower weight, heart failure, higher doses of inotropes, pulmonary hypertension, respiratory infections and delayed sternal closure were independent predictors of PMV in multivariate analyses.The results of this study indicated that PMV predictors could be specific for each center and a good administration program is needed for each pediatric cardiac surgery center for the preoperative management of patients undergoing congenital heart surgeries.
: Pulmonary hypertension is usually
classified as primary (idiopathic) or
secondary.1 It is now clear, however, that
there are conditions within the category of
secondary pulmonary hypertension that
resemble primary pulmonary hypertension
in their histopathological features and their
response to treatment. For this reason,
the World Health Organization (WHO)
classified pulmonary hypertension into
five groups on the basis of mechanisms,
rather than associated conditions.
In 2003, the World Health Organization
revised the classification of PAH into
5 categories based in part on etiology:
pulmonary arterial hypertension, pulmonary
venous hypertension, pulmonary
hypertension associated with hypoxemia,
pulmonary hypertension resulting from
chronic thrombotic or embolic disease,
and miscellaneous. 2 Group I of the WHO
classification, designated pulmonary
arterial hypertension, is the principal focus
of this review.
Pulmonary arterial hypertension is
defined as a sustained elevation of
pulmonary arterial pressure to more
than 25 mm Hg at rest or to more than
30 mm Hg with exercise, with a mean
pulmonary-capillary wedge pressure and
left ventricular end-diastolic pressure of
less than 15 mm Hg.3Diagnosis of PPH
one must excludes all secondary cause of
PHT and demonstrate normal pulmonary
wedge pressure. 2,3
Pathophysiology:
The histopathology of pulmonary vascular
disease has been classified in two phases,
early description in the 1950s (the Heath-
Edwards classification) and an extension
of this by Rabinovitch et al. 2.3Although pulmonary vascular resistance(
PVR = mPAP _ mPCWP/Q p) may be as
high as 8-10 Wood units immediately after
birth, it normally falls rapidly through
the first week of life. By 6-8 weeks,
pulmonary vascular resistance usually
has reached a normal adult level of 1-3
Wood units. In an infant, although the
pulmonary pressure is at systemic levels,
the pulmonary vascular resistance is low
and there is pulmonary over circulation.
2.3,23,26,28
Frequency data are difficult to confirm,
as there are no international registries
tracking the incidence and prevalence
of pulmonary hypertension. Elevated
pulmonary artery pressure in congenital
heart disease is caused by pulmonary over
circulation, pulmonary vasoconstriction,
and pulmonary vascular disease, either
alone or in combination.
Despite the spontaneous regression of many cases of coronary artery fistulas (CAFs), symptomatic patients or cases with severe shunting may require therapeutic interventions. In the present study, we aimed to assess the outcome of treatment of CAFs using interventional approaches.This retrospective cohort study was performed on 29 patients with CAFs that were referred to our tertiary center between 2009 and 2019. Baseline characteristics were collected by reviewing the hospital files, and the patients were followed up to assess long-term outcomes with a mean follow-up of 3.3 years.Overall, in 29 patients in our cohort study, 82.9% suffered from isolated CAFs and in the remaining cases, concurrent congenital abnormalities did exist. For treatment, coils (Cook, Pfm, Ev3) were used in 79.3%, ADO II(AGA) in 18.3%, vascular plug (AGA) in 3.4%, and a combination of coil/ vascular plug/amplatzer in 3.4%. Postoperative complications were reported in 4 patients as external iliac artery thrombosis, transient PSVT, ST-T wave changes and mild pericardial effusion that were all managed successfully with no adverse sequels. No coronary artery injury, device dislocation, dissection, ischemia or coronary dilatation occurred, and there was no death. As larger fistulas were treated by a retrograde approach through the right side of the heart, there was significant correlation between residual shunts and the mode of closure approach; the majority of the residual shunts occurred in patients in the retrograde approach group.Trans-catheter approach for treating CAFs leads to appropriate long-term outcome with minimal potential side effects.
Cyanotic congenital heart diseases are among the most serious anomalies among newborns. A rare type of this condition is direct communication between the right pulmonary artery and left atrium, which presents mostly in adolescence and adulthood. Large shunts, however, should be corrected as soon as possible, considering their potential to cause congestive heart failure. Two 2- and 13-year-old patients with this pathologic communication complained of exertional dyspnea and central cyanosis. Their physical exams were prominent, with a low oxygen saturation level. The diagnostic modalities used, electrocardiogram, chest X-ray, echocardiogram, CT scan, and selective angiography of the pulmonary arteries, showed right-to-left abnormal blood flow through the right pulmonary artery and left atrium shunt. Finally, both patients were treated successfully by a transcatheter occluder without any complications or follow-up complaints. The right pulmonary artery and left atrium abnormal congenital connections are rare causes of central cyanosis, mostly present with exertional dyspnea and cyanosis during adolescence or early adulthood. Transthoracic echocardiography, contrast-enhanced CT scans, and angiography of the pulmonary arteries make the diagnosis. The treatment has emerged during the last two decades, shifting from surgical treatment for severe cases to interventional percutaneous strategies, leaving the surgery for cases with no appropriate location for the application of interventional therapy. Considering the potentially life-threatening complications of the untreated right pulmonary artery and left atrium fistulas, such as thromboembolic events, early diagnosis is crucial. However, treating these patients is not always straightforward, and strategies should be selected based on the connecting fistula's anatomical features, location, size, and tortuosity. Although endovascular treatment is the preferred option, some patients need to be treated with surgical procedures due to their structural characteristics.
Introduction: Congenital portosystemic shunts constitute a rare disorder in childhood with a wide spectrum of symptoms and signs, one of which may be cyanosis. Case Presentation: We describe for the first time in Iran a 9-year-old boy with a portacaval shunt presenting with cyanosis. He was diagnosed, managed, and treated successfully. The postoperative period was complicated by portal vein thrombosis, which wasgradually resolved with appropriate treatment. Conclusions: Cyanosis is one of the hallmarks of cardiopulmonary disease; however, as was the case in our patient, a secondary cause of pulmonary disease should be considered. The portacaval shunt is a rare but important cause of cyanosis and should be considered in all patients in whom cyanosis is unexplained.
Background: Transcatheter stent implantation for native aortic coarctation (CoA) in pediatric patients is widely performed but data on mid and long-term results are scarce. The study aimed to determine the long-term safety and efficacy of transcatheter CoA stenting based on centrally adjudicated outcomes.Methods: This retrospective cohort recruited patients aged up to 15 years who had undergone de novo stenting for CoA or reCoA between 2006 and 2017. Both immediate and 5-year outcomes were investigated. Immediate outcomes (procedural and in-hospital) were retrieved from electronic records. The adjudicated rates of 5-year recoarctation, stent fractures, aneurysmal/pseudoaneurysmal formation, and all-cause mortality were reported as the long-term outcomes.Results: The study evaluated 274 patients, including 98 females (35.8%), with a median (interquartile range) age of 9 (6-12) years. Procedural success was achieved in 251 (91.6%) patients with procedural complications in 4 (1.4%) patients, including 1 stent migration (0.3%) and 3 (1.1%) small non-expanding non–flow-limiting aortic wall injuries. Major vascular access complications occurred in 18 (6.6%) patients, dominated by acute limb ischemia in 8 (2.9%) patients. Four (1.4%) cases of in-hospital mortality occurred. The 5-year cumulative incidence rates of stent fractures, recoarctation, and aortic aneurysmal/pseudoaneurysmal formation were 17/100 (17%), 73/154 (48%), and 8/101 (7.92%), respectively. Of 73 recoarctations, forty-seven patients were treated with balloon angioplasty, 15 underwent a second stent implantation procedure Five-year all-cause mortality occurred in 4/251 (1.6%) patients.Conclusions: Coarctoplasty with stents was safe and effective in our pediatric patient population during a five-year follow-up despite a high rate of recoarctation.
A 4-year-old boy developed dyspnea 18 months after attempted surgical patent ductus arteriosus closure using a clip that was inadvertently placed on the left pulmonary artery, followed by reoperation to repair the left pulmonary artery with a patch and re-close the ductus. Computed tomography angiography confirmed a large patent ductus arteriosus, left pulmonary artery occlusion, and patent left pulmonary veins. Therefore, a third surgery was performed for suture closure of the ductus and left pulmonary artery repair with an interposition tube graft. Follow-up by transthoracic echocardiography and computed tomography angiography showed good flow in the left and right pulmonary arteries.
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