Early postoperative physical therapy for improving short-term gross motor outcome in infants with cyanotic and acyanotic congenital heart disease

Purpose: We analysed the gross motor recovery of infants and toddlers with cyanotic and acyanotic congenital heart disease (CHD) who received early postoperative physical therapy to see whether there was any difference in the duration to recovery.

Methods: This study retrospectively evaluated the influence of early physical therapy on postoperative gross motor outcomes of patients with CHD. The gross motor ability of patients with cyanotic (n?=?25, average age: 376.4 days) and acyanotic (n?=?26, average age: 164.5 days) CHD was evaluated using our newly developed nine-grade mobility assessment scale.

Results: Physical therapy was started at an average of five days after surgery, during which each patient’s gross motor ability was significantly decreased compared with the preoperative level. Patients (who received early postoperative physical therapy) with cyanotic (88.0%) and acyanotic CHD (96.2%) showed improved preoperative mobility grades by the time of hospital discharge. However, patients with cyanotic CHD had a significantly prolonged recovery period compared to those with acyanotic CHD (p?Conclusions: Our findings suggested that infants with cyanotic CHD are likely at a greater risk of gross motor delays, the recovery of which might differ between infants with cyanotic and acyanotic CHD after cardiac surgery. Early postoperative physical therapy promotes gross motor recovery.

• Implications of Rehabilitation

• Infants and toddlers with cyanotic congenital heart disease are likely at greater risk of gross motor delays and have a prolonged recovery period of gross motor ability compared to those with acyanotic congenital heart disease.

• Early postoperative physical therapy for patients with congenital heart disease after cardiac surgery promoted gross motor recovery.

• The postoperative recovery period to preoperative mobility grade was affected by pre-, intra-, and postoperative factors.

• Rehabilitation experts should consider the risk of gross motor delays of patients with congenital heart disease after cardiac surgery and the early postoperative physical therapy to promote their gross motor recovery.

     

彩色多普勒超声心动图对新生儿先天性心脏病的诊断

目的 探讨彩色多普勒超声心动图对新生儿先天性心脏病早期诊断和鉴别的价值。方法 对因青紫、心脏杂音、呼吸困难、心衰、黄疸等新生儿650例患儿诊断，进行常规切面及剑下各切面彩色多普勒超声心动图检查、复杂琦形者按心脏三级顺序分段法检查并作出诊断。结果 检出先心病136便，其中以室间隔缺损、动脉导管未闭、房间隔缺损发生为首位，发绀型以大动永转位和法洛四联征为首位。结论 彩色多普勒超声心动图因其无创，为患儿提供了方便、快捷的手段，使先心病的早期准确诊断成为可能，尤其是引起新生儿死亡主要原因的紫绀型，早期诊断及鉴别诊断是治疗的关键，同时对治疗方案的选择及估计预后亦其有重要的价值。而非常绀型先心病的早期诊断，对于择期手术，预防感性心内膜炎，保证患儿的正常生长发育具有十分重要意义。     

Cardiac emergencies in the first year of life

Cardiac emergencies in the first year of life can be anxiety provoking for the health care provider. An understanding of the pathophysiology involved in the most common emergency department presentations is crucial to the development of appropriate treatment plans. This article discusses the most common causes of cyanotic and acyanotic heart disease in infants.     

Cardiac emergencies in infancy and childhood

There are relatively few situations where cardiac emergencies can occur in a child. A special focus is directed to the cyanotic newborn. In these cases a very specific treatment plan is necessary which is limited to a few dedicated centres. The importance lies in the timely diagnosis of a cyanotic heart defect and the rapid and safe transport from the referring hospital. An outline is given on the causes and treatment of congestive heart failure in infancy. Management guidelines are given for the most frequent types of cardiac arrhythmias and the few occasions, where an arrhythmia can lead to a real emergency, mostly in infants. To complete the list of the most frequent cardiac emergencies in pediatrics, pericardial effusion/tamponade is mentioned as well as the occurrence of cyanotic spells typically in the baby with Fallot's tetralogy.     

Isolated interrupted aortic arch in an adult: A case report

BACKGROUNDInterrupted aortic arch (IAA) is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries. It is usually found within a few hours or days of birth. Without surgery, the chances of survival are low. If IAA patients have an effective collateral circulation established, they can survive into adulthood. However, IAA in adults is extremely rare, with few reported cases.CASE SUMMARYA 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion. She had cyanotic lips and clubbing of the fingers. A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery. There was an abnormal 9 mm passage between the descending aorta and pulmonary artery. The ventricular septal outflow tract had a 14 mm defect. Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch. The diagnoses were ventricular septal defect, patent ductus arteriosus, and definite interruption of the aortic arch. Although surgical correction was recommended, the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure. Her condition has been stable for 12 mo of follow-up.CONCLUSIONAlthough rare, IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.     

体外受精-胚胎移植胎儿先天性心脏病的超声表现及分类特征

目的探讨体外受精-胚胎移植（IVF-ET）妊娠的心脏病胎儿超声心动图特征及分类情况的特殊性。方法对2011年1月至2013年6月孕18-24周在南京军区福州总医院行胎儿超声心动图检查的IVF-ET妊娠孕妇1129例,分析IVF-ET妊娠胎儿先天性心脏病超声表现及分类特征,并对部分检出胎儿心脏病的孕妇选择性进行羊水穿刺术,追踪所有入选孕妇的妊娠过程及胎儿临床结局。结果1129例接受IVF-ET的孕妇中单胎妊娠953例、双胎妊娠176例,共检测胎儿1305个,其中超声心动图诊断胎儿心脏畸形15例（1.1%,15/1305）,临床确诊15例。IVF-ET心脏畸形胎儿超声心动图表现：（1）法洛四联症5例,超声心动图示胎儿主动脉增宽伴骑跨,室间隔缺损,肺动脉狭窄,右心室肥厚。（2）左心发育不良综合征1例,超声心动图示胎儿左心明显缩小,二尖瓣闭锁,右心扩大,肺动脉扩张。（3）完全型房室间隔缺损1例,超声心动图示胎儿心内膜正常十字交叉结构消失,房室共瓣。（4）大动脉共干Ⅰ型1例,超声心动图示胎儿心底部仅见1条大血管,短小的肺动脉主干自大动脉发出,并见室间隔缺损。（5）主动脉狭窄及缩窄各1例,超声心动图示1例胎儿主动脉瓣增厚,升主动脉增宽;1例胎儿主动脉峡部狭窄伴狭窄后扩张。（6）肺动脉狭窄2例,超声心动图示胎儿肺动脉瓣狭窄伴狭窄后肺动脉扩张。（7）室间隔缺损3例,超声心动图示胎儿室间隔连续中断,断端边缘回声增强。超声漏诊胎儿室间隔小缺损1例,为出生后超声心动图检查发现。结论 IVF-ET妊娠胎儿较自然妊娠胎儿先天性心脏病的发生率高,IVF-ET妊娠胎儿与自然妊娠胎儿先天性心脏病的超声心动图表现相同。其中以法洛四联症和室间隔缺损最多见。对IVF-ET妊娠胎儿行超声心动图检查能全面系统评价IVF-ET妊娠胎儿心脏异常的情况,有重要临床指导意义。     

Sevoflurane induction in cyanotic and acyanotic children with congenital heart disease

Introduction The aim of the study was to investigate the induction characteristics of sevoflurane in cyanotic and acyanotic children with congenital heart disease compared with healthy controls in a prospective, controlled, non-blinded study at a university hospital. Methods After placement of standard noninvasive monitors, inhaled induction of anaesthesia was achieved via a facemask by using 7% sevoflurane in 100% oxygen, in all 3 groups (control group, n=16; acyanotic group, n=32; cyanotic group, n=30). Three minutes after induction, tracheal intubation was performed without a muscle relaxant. In each case, the time from mask application to loss of eyelash reflex and to centralisation of the pupils, heart rate, blood pressure and oxygen saturation was recorded. After intubation, intubating conditions were graded and incidences of airway obstruction recorded. The demographic data was similar between the 3 groups except for the sex ratio in Group 1 compared with the other groups (P=0.005). Results The durations to loss of eyelash reflex were found to be significant between the patients with acyanotic and cyanotic heart disease (P=0.044). Centralisation of the pupils, heart rate and blood pressure results, as well as the incidences of airway obstruction and intubation conditions, were similar between the groups (P>0.05). In addition, oxygen saturation was significantly lower in the cyanotic group at all stages (P<0.01). Conclusion Induction characteristics of sevoflurane in cyanotic and acyanotic children with congenital heart disease and in healthy children are similar. Sevoflurane induction is an effective and well-tolerated technique for cyanotic and acyanotic children with congenital heart disease.     

Contemporary thinking for congenital heart disease

Understanding congenital heart disease (CHD) in terms of three physiologic categories provides the pediatric nurse with practical, effective interventions for sick children. Children with increased pulmonary blood flow require support for congestive heart failure (CHF) symptoms including medications, increased caloric intake, and organization of care. Children with significantly decreased pulmonary blood flow need surgical intervention to create a path for pulmonary blood supply. Children with obstruction to systemic blood flow require support for both CHF symptoms and poor ventricular contractility. Shifting the focus of understanding from cyanotic and acyanotic defects to these three physiologic categories improves the pediatric nurse's capability to plan and care for children with CHD.     

What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly?

Adults with congenital heart disease (CHD) represent a growing population of patients thanks to the medical and surgical advances which enable at least 85% of children to survive to adulthood. These advances may create quality-of-life (QoL) issues not previously considered. The purpose of this cross-sectional study of 124 adults with CHD was to describe their QoL as a basis for providing appropriate information, counseling, and anticipatory guidance. Thirteen patients had single ventricle physiology (SVP), 43 had cyanotic lesions with 2 ventricle repairs, and 68 had acyanotic CHD. On the basis of Sickness Impact Profile (SIP) scores, individuals with SVP had worse QoL than did those with cyanotic lesions (with 2 ventricle repairs) and acyanotic anomalies (SIP = 9.98 vs 4.61 and 3.76). SIP scores were statistically significantly different between those with SVP and those with acyanotic anomalies (P = .02). For all groups, the areas of life most affected were work and sleep and rest. Participants with SVP saw themselves as having the poorest QoL.     

非紫绀型先天性心脏病儿童的词语理解能力研究

目的探讨非紫绀型先天性心脏病(CHD)儿童词语理解能力的发育状况。 方法选择2~4岁非紫绀型CHD患儿64例（先心病组）和健康儿童60例（对照组）。采用修订的皮博迪图片词汇测验——儿童词语理解能力测验（VCTC）评定工具评估其词语理解能力,并分析房间隔缺损（ASD）、室间隔缺损（VSD）、动脉导管未闭（PDA）3种非紫绀型CHD及不同性别患儿的词语理解能力得分差异。 结果非紫绀型CHD患儿词语理解能力迟缓的发生率为20%,健康儿童词语理解能力迟缓的发生率为5%,差异有统计学意义(P<0.05)。非紫绀型CHD患儿的词语理解能力得分低于健康儿童,差异有统计学意义(P<0.05)。ASD、VSD、PDA患儿之间的词语理解能力得分差异无统计学意义(P>0.05);不同性别的非紫绀型CHD患儿词语理解能力得分差异无统计学差异 (P>0.05)。 结论非紫绀型CHD患儿存在词语理解发育迟缓倾向,非紫绀型CHD的类型、性别并非是影响患儿词语理解能力发育的重要因素。     

Educational needs of adults with congenital heart disease

Adults with congenital heart disease represent a new and growing population of patients who pose a challenge to both medicine and nursing. The purpose of this study was to identify the educational needs of adults with congenital heart disease. The study used a prospective, cross-sectional, case-control study design and enrolled 124 adults with congenital heart disease and 124 matched healthy controls. Adults with congenital heart disease were more likely to report living at home with parents and less likely to be employed compared with healthy controls. When those adults with acyanotic and cyanotic congenital heart disease were compared, acyanotic adults were twice as likely to be employed. Additionally, several areas of educational needs were identified in this study for adults with congenital heart disease.     

New directions in device therapies among children and adults with congenital heart

Innovative, nonthoracotomy, catheter-delivered therapies have redefined the approach to and treatment of congenital heart defects. Starting in the 1960s with the creation of an opening in the atrial septum to permit effective blood mixing and improve oxygen saturation in cyanotic infants, interventional cardiac procedures continue to replace many of the time-honored surgeries that were the mainstay of repair or correction for infants and children with heart defects. Now as those children reach adulthood and still require modifications of their defects, catheter-based interventions are becoming more important. This article examines some of the more recent applications of device therapy currently available to patients with congenital heart, including heart failure, septal defects, vascular problems and heart valves. Device use in deference to surgery, risks and benefits as well as complications associated with such catheter-delivered therapies are discussed.     

Deadly defects. Managing the pediatric patient with a congenital heart defect

CHD-specific emergencies may be managed quite effectively in the prehospital environment. The key to successful prehospital management of CHDs is identifying the cardiac anomaly, obtaining an effective history and physical assessment, and providing supportive care. When obtaining a history, it is important to remember that parents know their children and their children's diseases very well. An EMS provider who ignores information from a parent is doomed to failure. Realizing that most EMS providers are not well-versed in the various CHDs and are not likely to have a great deal of experience in working with CHD patients, conferring with medical control prior to initiation of any treatments is strongly encouraged. Finally, remember that patients with CHDs are sick and have traditionally been sick for quite a while. Because of the chronic illness, it is easy to focus on the congenital defect when called to the scene for an ill child and neglect the potential for a noncardiac-related pathology. Although the most commonly seen congenital heart defects have been reviewed here, there are others that have not been addressed. Garnering an understanding of each individual defect is not nearly as important as understanding the difference between cyanotic and acyanotic defects. Prehospital care for the CHD patient is primarily supportive, but, in certain cases, may require substantial intervention. EMS providers should not be concerned with diagnosing specific defects, but should be aware of the global effects that various defects have on normal perfusion. EMS providers' comprehension of the pathophysiology of CHDs and prompt actions will play a vital role in the outcome of the acutely ill CHD patient.     

New directions in device therapies among children and adults with congenital heart

Innovative, nonthoracotomy, catheter-delivered therapies have redefined the approach to and treatment of congenital heart defects. Starting in the 1960s with the creation of an opening in the atrial septum to permit effective blood mixing and improve oxygen saturation in cyanotic infants, interventional cardiac procedures continue to replace many of the time-honored surgeries that were the mainstay of repair or correction for infants and children with heart defects. Now as those children reach adulthood and still require modifications of their defects, catheter-based interventions are becoming more important. This article examines some of the more recent applications of device therapy currently available to patients with congenital heart, including heart failure, septal defects, vascular problems and heart valves. Device use in deference to surgery, risks and benefits as well as complications associated with such catheter-delivered therapies are discussed.     

经腹部超声心动图在孕早期胎儿先天性心脏病诊断中的应用价值

目的探讨经腹部超声心动图在孕早期胎儿先天性心脏病（CHD）诊断中的应用价值。 方法对2012年6月至2015年4月在南京军区福州总医院产前检查的1 288例高危孕妇孕早期（孕13周）胎儿行超声心动图检查,分析孕早期胎儿CHD超声表现及其特征,追踪所有入选孕妇的妊娠过程及胎儿临床结局。 结果经腹部超声心动图在1288例胎儿中诊断CHD 16例（1.2%,16/1288）,其中复杂型CHD 11例,单纯型CHD 5例。孕早期胎儿CHD超声心动图表现：（1）法洛四联症2例,超声心动图显示主动脉轻度增宽,未见明显骑跨,室间隔缺损,肺动脉狭窄,远端显示不清。（2）左心发育不良综合征2例,孕13周超声心动图显示左心明显缩小,右心扩大,二尖瓣闭锁,室间隔缺损,主动脉轻度狭窄伴血流速度加快,肺动脉增宽。（3）大动脉共干I型2例,超声心动图显示心底部仅见1条大血管,未见明显分支血管,并见室间隔缺损。（4）完全型房室间隔缺损4例,超声心动图显示心内膜正常十字交叉结构消失,房室共瓣。（5）单心室1例,超声心动图未见室间隔回声,仅见一单心室。（6）主动脉缩窄1例,孕13周超声心动图显示心内结构正常,主动脉内径正常;孕16周超声心动图显示左心偏小,主动脉峡部轻度狭窄。（7）室间隔缺损4例,超声心动图显示室间隔连续中断,断端边缘回声增强。1例小的室间隔缺损误诊,漏诊室间隔缺损和永存左上腔静脉各1例。超声诊断CHD后孕妇选择终止妊娠11例,出生5例,随访结果与产前诊断均相符。 结论经腹部超声心动图可早期诊断多数胎儿CHD,尤其表现为四腔心异常的CHD,但对CHD异常大血管起源、分支及走向的显示存在一定困难,有待中孕期进一步确诊,早孕期经腹部超声心动图检查有重要临床应用价值。     

Congenital heart disease

Pediatric congenital heart disease comprises a wide spectrum of structural defects. These lesions present in a limited number of ways. An infant presenting with profound shock, cyanosis, or evidence of congestive heart failure should raise the suspicion of congenital heart disease. Although most congenital lesions are diagnosed in utero, the emergency physician must be aware of these cardinal presentations because many patients present in the postnatal period around the time that the ductus arteriosus closes. Aggressive management of cardiopulmonary instability combined with empiric use of prostaglandin E(1) and early pediatric cardiology consultation is essential for positive outcomes.     

Indications for pediatric cardiology evaluation

Heart disease in infants and children may often lead to rapid decompensation. Early recognition by the primary physician is essential, since modern medicine and surgery can cure many heart defects. Main clinical signs are cyanosis, heart failure and/or abnormal auscultatory findings. Cyanosis appears shortly after birth or in small infants. In the newborn, cyanosis due to heart disease must be differentiated from pulmonary problems, sepsis or persistent pulmonary hypertension. Heart failure occurs more often in non cyanotic heart defects, mainly with left to right shunts. The main symptoms and signs of heart failure in the infant are briefly discussed. Abnormal auscultatory findings, usually murmurs, are the most common reason for referral to the paediatric cardiologist. However, most murmurs are "innocent" or "functional"; clues to their recognition are given. The present short review should help the primary physician to recognize signs of heart defects in infants and children so as to proceed with timely referral to the specialist.     

Patient after correction Taussig‐Bing anomaly with severe neo‐aortic regurgitation after sudden cardiac arrest

The Taussig‐Bing anomaly is a rare cyanotic congenital heart defect treated surgically in the early infancy. The preferred repair procedure is an arterial switch operation combined with ventricular septal defect closure. Despite promising long‐term functional outcomes and survival benefits, neo‐aortic dysfunction and arrhythmias might be relevant complications.     

Permanent Cardiac Pacing After Open Heart Surgery: Congenital Heart Disease

A retrospective review of 6,004 patients who underwent open repair of congenital heart defects revealed that 132 patients (2.2%) required permanent cardiac pacing postoperatively. The indications for pacing were early atrioventricular (AV) block in 55%, late onset AV block in 31%, and sick sinus syndrome in 14%. A ventricular septal defect (VSD) was the most common congenital anomaly present alone or in association with other lesions in 67% of the patients. Atrial surgery accounted for 21% of the patients requiring pacing. Ten-year patient survival was found to be 66% (+/- 6%). Thirty-five percent of the deaths were sudden and unexpected, presumably due to an arrhythmia. Reoperation for pacing system failure has occurred too frequently (12% per year). The most common causes for reoperation were battery failure (44%) and exit block (25%).     

Caring for patients with lesions increasing pulmonary blood flow

The most common congenital cardiac defect is VSD. This malady accounts for 20 to 30 per cent of all congenital cardiac defects and is representative of a cardiac lesion that increases pulmonary blood flow. Although lesions, which increase pulmonary blood flow, may vary in incidence, they frequently have common symptomatology. Over time, congestive heart failure becomes a problem. Poor respiratory status leads to weight loss, poor feeding, and failure to thrive. If unrepaired, the child often presents with cyanosis and tachypnea. The history may include frequent respiratory infections, exercise intolerance, generalized malaise, or fatigue. In spite of poor weight gain, the child may be edematous and have a large liver. Definitive diagnosis of each lesion may be made by echocardiogram, cardiac catheterization, or both. With these data and a detailed history, treatment and management decisions are determined. In most cases, as the child gets older, symptoms become more evident. This is the result of high pulmonary pressure. High pressure over time causes a thickening of the alveolar tissue, which decreases the permeability of the alveolar membranes for gas exchange. Lung changes can become irreversible, but it is unusual for irreversible changes to occur before 1 year of age. All the lesions described in this article are amendable to primary repair before 1 year of age, affording the best functional results. Postoperative nursing care includes management of persisting CHF and PVR while maintaining adequate cardiac output. Many factors, including electrolyte balance, nutritional status, conduction disturbances, stress, and parental anxiety, influence the management of these infants. The outcome depends greatly on the assessment skill of a highly competent cardiac intensive care nurse and an environment conducive to collaborative practice.