Balloon Valvuloplasty for Critical Pulmonary Valve Stenosis in a Premature Infant

Successful balloon valvuloplasty for critical pulmonary valve stenosis is described in an 800-g infant. A modified catheter was required to cross the valve in the smallest child known to undergo this procedure.     

Balloon Valvuloplasty of Critical Pulmonary Valve Stenosis in a Premature Neonate

Balloon dilatation of critical pulmonary valve stenosis in neonates with a weight of less than 2.5 kg is associated with specific problems, including temperature loss during the procedure, venous access, and problems related to the small size of the cardiac structures. We report our experience with balloon valvuloplasty in a premature newborn weighing 1.22 kg. Venous access was gained with a 4 French sheath, and balloon dilatation was performed with a 3.5 French 7-mm balloon catheter. Temperature loss of the baby was avoided by puncturing the femoral vein prior to the procedure on a neonatal open care system, wrapping the child in cotton, and covering the extremities with aluminium foil. The good result in our patient demonstrates that balloon valvuloplasty is a therapeutic option for treatment of critical pulmonary stenosis in premature infants.     

Intervention in Patients with Critical Pulmonary Stenosis in the Ductal Stenting Era

We aimed to assess early and midterm outcomes of balloon valvuloplasty (BVP) procedure in patients with critical pulmonary stenosis (CPS) and to describe the predictors of the need for additional pulmonary flow and reintervention in this subgroup of patients. From 2005 to 2014, 56 neonates were diagnosed with CPS and were included in this study. All echocardiographic, catheterization and angiographic data obtained prior to the initial BVP and at follow-up were reviewed. BVP was successful in 55 neonates (98 %). Twenty-one neonates needed pulmonary blood flow augmentation after BVP (38 %). Ductal stenting (DS) was performed in 20. The patients’ mean tricuspid valve (TV) annulus diameter was 10.4 ± 2 mm, and the Z score was ?1.29 ± 1 (?3.7 to 0.78). The mean pulmonary valve (PV) annulus diameter was 6 ± 0.9 mm, and the Z score was ?1.74 ± 1 (?4.34 to 0.05). A transcatheter or surgical reintervention was performed in 11 patients. A TV Z score < ?1.93 SD predicted the need for pulmonary blood flow augmentation after a successful BVP, with a sensitivity of 63.2% and a specificity of 84.4%. A PV Z score < ?1.69 SD predicted the need for pulmonary flow augmentation, with a sensitivity of 74 %. The presence of bipartite RV was found to be a significant predictor of the need for reintervention (odds ratio 9.6). Our study showed the excellent immediate outcomes of BPV and DS in a pure cohort of patients with CPS. Prophylactic DS in selected cases seems reasonable and safe.     

Right Ventricular Myocardial Sinusoidal–Coronary Artery Connections in Critical Pulmonary Valve Stenosis

Ventriculo-coronary arterial connections are frequently observed in pulmonary atresia with intact interventricular septum. Sustained right ventricular hypertension during cardiac morphogenesis is thought to be responsible for persistence of myocardial sinusoidal–coronary artery connections. Considering an unusual observation of ventriculo-coronary arterial connections in a patient with critical pulmonary valve stenosis, we speculate that sinusoidal connections in pulmonary atresia with intact ventricular septum occur not only because it is the only exit for blood from the blind right ventricle, but also because of intrinsic altered morphogenesis of the right ventricle myocardium.     

Percutaneous Balloon Valvuloplasty of Both Pulmonary and Aortic Valves in a Neonate with Pulmonary Atresia and Critical Aortic Stenosis

We report a case of hydrops fetalis originating from critical aortic stenosis and pulmonary atresia with intact ventricular septum that was diagnosed in utero by echocardiography. We performed a percutaneous balloon valvuloplasty of the bilateral semilunar valves on the 2nd day after birth. We used a stiff-ended guidewire to perforate the pulmonary valve. After valvuloplasty, the aortic valve pressure gradient decreased from 55 to 25 mmHg. The procedure was successful, and the patient’s heart condition improved.     

Aneurysm of the Membranous Septum in Critical Pulmonary Stenosis: Spontaneous Rupture After Balloon Dilatation

We report a case of critical pulmonary valve stenosis in which congenital aneurysm of the membranous septum ruptured spontaneously after balloon dilatation of the pulmonary valve. It is considered that the chronic mechanical trauma with phasic protrusion and collapse of the aneurysm during the cardiac cycles was responsible for an aneurysm rupture.     

CHARGE-Association with Pulmonary Stenosis

CHARGE-association was proposed by Pagon et al. (1) as an acronym for a malformation syndrome including Coloboma, Heart disease, Atresia choanae, Retarded growth and development and/or CNS anomalies, Genital anomalies and/or hypogonadism and Ear anomalies and/or deafness. More than 60 cases have been reported with four or more of the seven defects (2). Associated anomalies may appear but many of the features are midline facial or central nervous system defects. At least two separate genetic forms may exist even if sporadic cases are most frequent. This report concerns a boy with heart defects not previously described in the complete CHARGE-association.     

Exercise Testing in Children with Pulmonary Valvar Stenosis

Pulmonary valvar stenosis with intact ventricular septum is a common anomaly. This lesion poses a fixed obstruction to the right ventricular outflow. The right ventricle ejects the entire cardiac output across the stenotic valve. Right ventricular systolic pressure and oxygen demand are increased at rest and more so with exercise. Exercise tolerance in children and adults with mild valvar pulmonary stenosis is nearly normal, but is diminished in those with moderate and severe stenosis, indicating impaired ability to sustain adequate cardiac output. Following relief of stenosis, cardiac performance improves in children, but remains abnormal in adults. This appears to be related to postoperative resolution of right ventricular hypertrophy in children, whereas myocardial fibrosis may explain the lack of improvement in adults.     

Long-term Results of Balloon Pulmonary Valvuloplasty in Children with Congenital Pulmonary Valve Stenosis

Objective

Immediate, short and midterm outcome of balloon pulmonary valvuloplasty are well known, but there is limited information on long term results. We report long term results of 2–13. 5 years follow up of balloon pulmonary valvuloplasty in children.

Methods

From June 1998 to January 2012 sixty consecutive patients (33 females, 27 males) with moderate to severe valvar pulmonary stenosis (right ventricular to pulmonary artery pressure gradient greater than 50 mmHg) were considered for balloon valvuloplasty. The gradient was measured pre and immediately post–valvuloplasty at catheterization, and then by echocardiography at follow up. Follow up studies were performed 2–13.5 years (mean±SD; 7.1±2.5 years, median: 5.5 years) after procedure, by Doppler echocardiography in all patients and catheterization and angiography in two patients.

Findings

Balloon pulmonary valvuloplasty BPV was successful in 53 of 60 (88.3%) patients whereas surgical valvotomy was necessary in 6 to 60 (10%). There was one immediate death due to perforation of the right ventricular outflow tract. Pulmonary valve systolic pressure gradient decreased from 83.3±32.1 to 19.3±14.2 mmHg immediately after BPV and to 12.3±6.6 mmHg at late follow up (P<0.001). Pulmonary insufficiency was noted in 20 (38%) patient at short–term, but it was demonstrated in 17 (32%) at late follow up. A second valvuloplasty was performed in two (3.8%) patients presenting with re-stenosis.

Conclusion

The short, intermediate and long-term outcomes of pulmonary balloon valvuloplasty in children are excellent. Therefore it can be considered as the treatment of choice for children with pulmonary valve stenosis.     

经皮球囊肺动脉瓣成形术治疗小儿肺动脉狭窄的疗效

目的观察经皮球囊肺动脉瓣成形术(PBPV)治疗肺动脉瓣狭窄(PS)患儿的术后疗效及安全性。方法对本院2005-2008年采用PBPV治疗的PS患儿54例进行分析。首选右心导管分别测量患儿右心房、右心室及肺动脉压力,然后单球囊法扩张其肺动脉瓣,术后采用超声心动图评价疗效。结果右室收缩压:PS患儿术前(65.30±25.40)mmHg(1 mmHg=0.133 kPa),术后(34.20±10.08)mmHg;跨肺动脉瓣压力阶差(PG):PS患儿术前(50.30±20.01)mmHg,术后1 d(19.96±6.95)mmHg,术后1个月(19.40±6.87)mmHg;术后1 d PG<20 mmHg达89.7%,术后1个月PG<20 mmHg达92.1%。PS患儿均未观察到并发症发生,术中失血量少,无输血,术后生命体征平稳。术后1个月经超声心动图随访无再狭窄发生。术后心脏杂音减轻,震颤全部消失,心电图大部分正常。结论PBPV治疗PS是安全有效。病例选择、精确测定肺动脉瓣直径和选择大小合适的球囊是手术成功的重要环节。     

Cryo-Balloon Angioplasty for Pulmonary Vein Stenosis in Pediatric Patients

This study sought to determine the safety and effectiveness of cryo-balloon angioplasty (CbA) for pulmonary vein stenosis (PVS) in pediatric patients. Current therapy options for PVS are less than satisfactory due to recurrent progressive restenosis and neointimal proliferation. Catheterization database, hospital records, imaging studies, and pathologic specimens were reviewed for procedural-related and outcomes data in all patients who underwent pulmonary vein (PV) CbA using the Boston Scientific PolarCath Peripheral Dilation System between August 2006 and June 2009. Thirteen patients (19 PVs; median age 13 months [range 3.5 months to 18.5 years] and weight 7.9 kg [range 3.8 to 47.7]) underwent CbA. Mean PVS diameter after CbA increased from 2.19 (±0.6) to 3.77 (±1.1) mm (p < 0.001). Mean gradient decreased from 14 (±7.4) to 4.89 (±3.2) mm Hg (p < 0.001). Mean stenosis–to–normal vein diameter ratio increased from 0.52 (±0.15) to 0.89 (±0.33) (p < 0.001). Eight patients underwent repeat catheterization a mean of 5.6 months (±3.66) later. Improved PVS diameter was maintained in 2 PVs. Four veins had restenosis but maintained diameters greater than that before initial CbA. In 11 PVs, the diameter decreased from 4.28 (±1.14) to 2.53 (±0.9) mm (p = 0.001). Mean gradient increased from 3.55 (±3.0) to 14.63 (±9.6) mm Hg (p = 0.011). All vessels underwent repeat intervention with acute relief of PVS. Stroke occurred within 24 h of CbA in 1 patient. CbA of PVS is safe and results in acute relief of stenosis. However, CbA appears minimally effective as the sole therapy in maintaining long-term relief of PVS.     

Device Closure of an Atrial Septal Defect Following Successful Balloon Valvuloplasty in a Neonate with Critical Pulmonary Valve Stenosis and Persistent Cyanosis

Persistent cyanosis after successful balloon valvuloplasty for neonatal critical pulmonary valve stenosis is often related to poor right ventricular compliance and right-to-left shunting at the atrial level. A successful catheter closure of an atrial septal defect was performed with a dramatic increase in systemic oxygen saturation alleviating the need for a surgical systemic-to-pulmonary artery shunt.     

Catheterization Treatment of Stenosis and Hypoplasia of Pulmonary Arteries

Stenosis of pulmonary arteries is one of the most challenging problems requiring treatment in the care of patients with congenital and acquired cardiopulmonary disease. Surgical approaches have been met with difficulty over the years, and may themselves lead to further distortion of the treated arteries. Balloon dilation first came into use in the 1980s, and has proved moderately effective. Its use has been extended to proximal pulmonary valve stenosis in order to improve distal flow and artery growth in some variants of tetralogy of Fallot. More recently, the judicious application of stent implantation has improved the outlook for pulmonary artery stenosis. The etiology, treatment (with balloon dilation and stent placement), and prognosis of pulmonary arterial stenosis will be discussed.     

心电图在重度肺动脉瓣狭窄诊治中的价值

目的 探讨心电图在重度肺动脉瓣狭窄（PS）诊治中的应用价值。方法 结合心脏超声和心导管检查结果，对34例单纯性重度PS患儿（男21例，女13例；平均年龄3．5岁）经皮球囊肺动脉瓣成形术（PBPV）前后心电图进行分析；并与34例轻中度患儿心电图进行比较。结果 单纯重度PS的心电图电轴极度右偏、P波及VIR波振幅增高，与轻中度PS比较存在显著差异（P〈0．05）；V1T波振幅与重度PS的右心室压力、跨肺动脉瓣压力阶差呈直线正相关（r=0．81，0．82 Pα〈0．05）；PBPV不同疗效组间心电图比较无显著性差异（P〉0，05）；PBPV术后近期心电图随访显示，其在心电轴、V1R波的电压、V1T波异常方面均有显著好转（Pα〈0．05）。结论 心电图对PS严重程度具有一定的诊断价值；根据V1R波振幅可估测右心室压力和跨肺动脉瓣的压力阶差；心电图无法预测PBPV术的疗效，但对PBPV术后的随访有重要辅助作用。     

Congenital Pulmonary Vein Stenosis: Encouraging Mid-term Outcome

Congenital pulmonary vein stenosis (PVS) is a rare entity with limited outcome literature. Multiple interventional approaches have evolved including surgical and catheterization techniques. Our objective is to report our center experience and to compare short-term and mid-term outcomes among these therapeutic modalities. Retrospective study on 23 patients (n = 23) with PVS that required intervention over the last 13 years (2000–2013). Patients were divided into three groups based on type of initial intervention. Of these, 10 (43.5 %) had balloon angioplasty, 3 (13.0 %) had surgical dilation, and 10 (43.5 %) had surgical marsupialization. Mortality and number of re-interventions were our primary outcomes. Mean age at diagnosis was 10.9 ± 18.4 months. Mean age at initial intervention was 14.5 ± 18.0 months. Mean pre- and post-initial intervention PVS gradients were 9.2 ± 3.4 and 3.4 ± 2.2 mmHg, respectively. Mean survival time and re-intervention-free survival time were 4.8 ± 4.0 and 2.8 ± 3.4 years. No statistical significance was found between the interventions with respect to survival time (p = 0.52) and re-intervention free time (p = 0.78). High initial pre- and post-intervention gradients were significantly associated with re-intervention-free survival (p = 0.01 and p = 0.03, respectively). Patients with bilateral disease have increased mortality (p = 0.01) and decreased 5-year survival (p = 0.009) compared to patients with unilateral disease irrespective of type of intervention. No statistically significant difference in mortality or re-intervention rate was present among these different therapeutic modalities. This study has the longest follow-up so far reported in the current literature (58 months) with overall survival of 78 %.