Tetralogy of Fallot with Dextrocardia and Situs Inversus in a 7-Year-Old Boy

Tetralogy of Fallot is one of the most frequently diagnosed congenital cardiac abnormalities; however, its association with dextrocardia and situs inversus is reported only rarely. We report a case of these findings in a 7-year-old boy, describe the patient''s postoperative course, and review the medical literature relevant to these combined conditions.Key words: Cardiac surgical procedures, child, coronary vessel anomalies/complications, dextrocardia, heart defects/congenital, situs inversus, tetralogy of Fallot/complications/diagnosis/epidemiology/surgery, treatment outcomeTetralogy of Fallot (TOF) is the most frequently diagnosed cyanotic congenital heart defect. It is often associated with additional findings, such as secundum atrial septal defect or right aortic arch. Its association with complete situs inversus is rarely reported and can present technical challenges to intracardiac repair. We report the case of a child who had TOF with dextrocardia and situs inversus, discuss the patient''s postoperative course, and review the relevant medical literature.     

Deep hypothermic arrest in children undergoing heart surgery

Deep hypothermia with cold cardioplegia is a technique that increases the chances for successful palliative or reparative cardiac surgery for the critically ill infant or child with complex or severe cardiac anomalies. Perioperative nursing care is of vital importance to the child, family, and operative team. Nursing care measures include perioperative teaching as well as preoperative and postoperative physical assessment of the child and psychosocial assessment of the child and family unit. An understanding of the advantages and the potential complications of hypothermic techniques enhances the nurse's ability to carry out these nursing interventions.     

Management after childhood repair of tetralogy of Fallot

Opinion statement The four characteristic findings in tetralogy of Fallot (TOF) include the following: 1) a malaligned ventricular septal defect; 2) right ventricular outflow and/or pulmonary valve/artery stenosis or atresia; 3) a dextraposed overriding aorta; and 4) right ventricular hypertrophy. This article focuses solely on the outcomes and treatment of surgically corrected patients born with TOF. In the modern era, early surgical repair consisting of ventricular septal defect closure and alleviation of right ventricular outflow obstruction have gained favor over early palliation with an aortopulmonary shunt followed by intracardiac repair. Surgical outcomes are excellent and dramatically improve prognosis. However, these patients are not "cured" and are at significant risk of developing subsequent electrical and hemodynamic problems. An operated patient with TOF should be evaluated at regular intervals by a cardiologist trained in congenital heart disease; any symptoms suggestive of hemodynamic or electrical compromise should spur further investigation. Advances in imaging, medical therapy, electrophysiology, device/resynchronization therapy, and percutaneous intervention provide the clinician with a number of therapeutic options. Surgical techniques have changed significantly since the early intracardiac repairs of the 1960s and 1970s. The deleterious hemodynamic and electrical effects of pulmonary regurgitation and ventriculotomy scars have spurred efforts to ensure pulmonary valvular competence and minimize the extent of ventricular incisions. Since Blalock and Taussig’s first palliative shunt in 1945, the survival and quality of life for patients with TOF has improved dramatically; this is one of the great accomplishments for cardiovascular medicine in the 20th century. The 21st century promises further success with the advent of a myriad of technologic advancements.     

Prognosis of right bundle branch block and left anterior hemiblock after intracardiac repair of tetralogy of Fallot.

One hundred and forty-one survivors of intracardiac repair of tetralogy of Fallot (TOF), operated on between 1958 and 1972, were studied in order to document the incidence of right bundle branch block and left anterior hemiblock (RBBB and LAH) and to definite the late prognosis. RBBB and LAH occurred in 31 patients (22 per cent), all of whom have had complete follow-up. Transient complete heart block (CHB) occurred postoperatively in 2 patients; there were no other significant arrhythmias. Two late deaths have occurred, neither from arrhythmia (one from progressive congestive heart failure, and the other from attempted reclosure of a ventricular septal defect). The remainder of the patients are well an average of 76 months postoperatively (range, 144 to 12 months). The absence of late-onset CHB or sudden death in this series contrasts with the relatively high incidence of these events in some studies of RBBB and LAH after intracardiac repair of TOF. However, in those reports a history of transient postoperative CHB (indicative of trifascicular disease in this setting) can be found in 75 per cent of those who developed late-onset CHB or died suddenly. We conclude that the occurrence of RBBB and LAH after intracardiac repair of TOF does not of itself carry a bad late prognosis. The critical factor in a bad late prognosis in patients with ECG evidence of RBBB and LAH may be with ECG evidence of RBBB and LAH may be the history of transient postoperative CHB.     

Perioperative Care of Children with Tetralogy of Fallot

OPINION STATEMENT: Complete surgical repair of tetralogy of Fallot (TOF) around 3?months of age is the commonly undertaken management strategy in many centers and has excellent outcomes. Intervention at an earlier age, including the newborn period, may be required for children with symptoms. Early extubation from mechanical ventilation where possible may help improve outcomes for children undergoing complete repair of TOF.     

Neonatal survival: a call for action

To achieve the Millennium Development Goal for child survival (MDG-4), neonatal deaths need to be prevented. Previous papers in this series have presented the size of the problem, discussed cost-effective interventions, and outlined a systematic approach to overcoming health-system constraints to scaling up. We address issues related to improving neonatal survival. Countries should not wait to initiate action. Success is possible in low-income countries and without highly developed technology. Effective, low-cost interventions exist, but are not present in programmes. Specific efforts are needed by safe motherhood and child survival programmes. Improved availability of skilled care during childbirth and family/community-based care through postnatal home visits will benefit mothers and their newborn babies. Incorporation of management of neonatal illness into the integrated management of childhood illness initiative (IMCI) will improve child survival. Engagement of the community and promotion of demand for care are crucial. To halve neonatal mortality between 2000 and 2015 should be one of the targets of MDG-4. Development, implementation, and monitoring of national action plans for neonatal survival is a priority. We estimate the running costs of the selected packages at 90% coverage in the 75 countries with the highest mortality rates to be US4.1 billion dollars a year, in addition to current expenditures of 2.0 billion dollars. About 30% of this money would be for interventions that have specific benefit for the newborn child; the remaining 70% will also benefit mothers and older children, and substantially reduce rates of stillbirths. The cost per neonatal death averted is estimated at 2100 dollars (range 1700-3100 dollars). Maternal, neonatal, and child health receive little funding relative to the large numbers of deaths. International donors and leaders of developing countries should be held accountable for meeting their commitments and increasing resources.     

Continuum of care for maternal, newborn, and child health: from slogan to service delivery

The continuum of care has become a rallying call to reduce the yearly toll of half a million maternal deaths, 4 million neonatal deaths, and 6 million child deaths. The continuum for maternal, newborn, and child health usually refers to continuity of individual care. Continuity of care is necessary throughout the lifecycle (adolescence, pregnancy, childbirth, the postnatal period, and childhood) and also between places of caregiving (including households and communities, outpatient and outreach services, and clinical-care settings). We define a population-level or public-health framework based on integrated service delivery throughout the lifecycle, and propose eight packages to promote health for mothers, babies, and children. These packages can be used to deliver more than 190 separate interventions, which would be difficult to scale up one by one. The packages encompass three which are delivered through clinical care (reproductive health, obstetric care, and care of sick newborn babies and children); four through outpatient and outreach services (reproductive health, antenatal care, postnatal care and child health services); and one through integrated family and community care throughout the lifecycle. Mothers and babies are at high risk in the first days after birth, and the lack of a defined postnatal care package is an important gap, which also contributes to discontinuity between maternal and child health programmes. Similarly, because the family and community package tends not to be regarded as part of the health system, few countries have made systematic efforts to scale it up or integrate it with other levels of care. Building the continuum of care for maternal, newborn, and child health with these packages will need effectiveness trials in various settings; policy support for integration; investment to strengthen health systems; and results-based operational management, especially at district level.     

Alma-Ata: Rebirth and Revision 6 Interventions to address maternal, newborn, and child survival: what difference can integrated primary health care strategies make?

Several recent reviews of maternal, newborn, and child health (MNCH) and mortality have emphasised that a large range of interventions are available with the potential to reduce deaths and disability. The emphasis within MNCH varies, with skilled care at facility levels recommended for saving maternal lives and scale-up of community and household care for improving newborn and child survival. Systematic review of new evidence on potentially useful interventions and delivery strategies identifies 37 key promotional, preventive, and treatment interventions and strategies for delivery in primary health care. Some are especially suitable for delivery through community support groups and health workers, whereas others can only be delivered by linking community-based strategies with functional first-level referral facilities. Case studies of MNCH indicators in Pakistan and Uganda show how primary health-care interventions can be used effectively. Inclusion of evidence-based interventions in MNCH programmes in primary health care at pragmatic coverage in these two countries could prevent 20-30% of all maternal deaths (up to 32% with capability for caesarean section at first-level facilities), 20-21% of newborn deaths, and 29-40% of all postneonatal deaths in children aged less than 5 years. Strengthening MNCH at the primary health-care level should be a priority for countries to reach their Millennium Development Goal targets for reducing maternal and child mortality.     

Right ventricular outflow tract stenting in tetrology of fallot with restrictive ventricular septal defect

A 6-month-old child with worsening cyanosis from birth, hypercyanotic spells and failure to thrive, was admitted with severe desaturation. Systemic saturation was < 56%, and echocardiography revealed severe infundibular pulmonary stenosis with normal pulmonary valve and a restrictive ventricular septal defect. The child underwent percutaneous stenting of the right ventricular outflow tract (RVOT), with good reduction in gradient and clinical stabilization. The child is being followed up for elective intracardiac repair.     

Patient- and family-centered care and the role of the emergency physician providing care to a child in the emergency department

Patient- and family-centered care (PFCC) is an approach to health care that recognizes the role of the family in providing medical care, encourages collaboration between the patient, family, and health care professionals; and honors individual and family strengths, cultures, traditions, and expertise. Although there are many opportunities for providing PFCC in the emergency department, there are also challenges to doing so. The American Academy of Pediatrics and American College of Emergency Physicians support the following: promoting patient dignity, comfort, and autonomy; recognizing the patient and family as key decision makers in the patient's medical care; recognizing the patient's experience and perspective in a culturally sensitive manner; acknowledging the interdependence of child and parent as well as the pediatric patient's evolving independence; encouraging family member presence; providing information to the family during interventions; encouraging collaboration with other health care professionals; acknowledging the importance of the patient's medical home; and encouraging institutional policies for PFCC.     

Complete Repair of Tetralogy of Fallot with Absent Pulmonary Valve Including the Role of Airway Stenting

Abstract Tetralogy of Fallot (TOF) with absent pulmonary valve (APV) represents an extreme form of tetralogy where pulmonary insufficiency and mild annular stenosis often results in massive pulmonary arterial (PA) dilatation. The aneurysmal left and right PAS often compress the adjacent trachea and bronchi, leading to airway obstruction and respiratory failure in infancy. Between 1991 and 1997, 11 patients underwent a single stage repair of TOF and APV using a valved (10 patients) or nonvalved (1 patient) homograft conduit and PA reduction arterioplasty. There was one (1/11 [9.1%]) perioperative and one (9.1%) late death. Both deaths were related to airway complications. Morbidity associated with postoperative respiratory complications and ventilator-dependency due to underlying tracheobronchomalacia is an important problem. Intermediate follow-up shows a high incidence of reintervention for conduit stenosis and/or insufficiency and tracheobronchial compression. These infants also required multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Stenting of the right and left main bronchi with balloon expandable metallic stents is a new experimental therapy that has been useful in two recent patients with respiratory failure despite satisfactory intracardiac repair. It may provide an attractive alternative therapy to prolonged mechanical ventilation with positive end expiratory pressure in patients with severe tracheobronchomalacia. Complete repair with a valved homograft conduit and reduction pulmonary arterioplasty in infancy at the time of diagnosis is the procedure of choice for infants with TOF with APV. With this approach the patient outcome is essentially determined by their airway status and airway management.     

Development of a Double-Chambered Right Ventricle After Repair of Tetralogy of Fallot

Objectives. We sought to determine the frequency, etiology and progressive nature of midcavity obstruction in patients after primary repair of tetralogy of Fallot (TOF).Background. Midcavity obstruction (double-chambered right ventricle [DCRV]) represents a significant portion of reoperations in patients who have had TOF repair. This group is still poorly defined.Methods. A retrospective review of clinical, echocardiographic and catheterization data for all patients with TOF who later underwent reoperation for DCRV was performed.Results. Between 1973 and 1995, 552 children <2 years of age underwent primary TOF repair (median age 6.7 months). Long-term follow-up (median 50 months) was available in 308 children. Of these, 17 children subsequently developed DCRV requiring reoperation. The median age at initial operation was 7.9 months. During a median follow-up interval of 43.2 months, murmur intensity increased in all patients, and the average subpulmonary gradient at catheterization increased from 24 ± 10 to 80 ± 27 mm Hg in seven children (p = 0.002) and at Doppler echocardiography from 14 ± 16 to 89 ± 18 mm Hg in five children (p = 0.002). Before reoperation, 6 of the 17 children were symptomatic. During the operation (median age 55.4 months), obstruction was relieved by incision of hypertrophied anomalous muscle bundles in all 17 patients, with prominent fibrosis noted in 8 patients. Excessive septal and parietal hypertrophy was noted in one child. No new transannular patches were required. Recurrent obstruction has reappeared in 3 of these 17 children during follow-up.Conclusions. DCRV is a medium-term complication of TOF repair in infants, with a minimal incidence of 3.1% (95% CI 1.8% to 4.9%). The condition is progressive and is due to anomalous muscle bundle hypertrophy or fibrosis, or both, which may represent displaced insertion of a moderator band. Further reobstruction does occur; continued careful follow-up is therefore essential.     

Integrating health interventions for women, newborn babies, and children: a framework for action

For women and children, especially those who are poor and disadvantaged, to benefit from primary health care, they need to access and use cost-effective interventions for maternal, newborn, and child health. The challenge facing weak health systems is how to deliver such packages. Experiences from countries such as Iran, Malaysia, Sri Lanka, and China, and from projects in countries like Tanzania and India, show that outcomes in maternal, newborn, and child health can be improved through integrated packages of cost-effective health-care interventions that are implemented incrementally in accordance with the capacity of health systems. Such packages should include community-based interventions that act in combination with social protection and intersectoral action in education, infrastructure, and poverty reduction. Interventions need to be planned and implemented at the district level, which requires strengthening of district planning and management skills. Furthermore, districts need to be supported by national strategies and policies, and, in the case of the least developed countries, also by international donors and other partners. If packages for maternal, newborn and child health care can be integrated within a gradually strengthened primary health-care system, continuity of care will be improved, including access to basic referral care before and during pregnancy, birth, the postpartum period, and throughout childhood.     

Differential Sex Learning in Early Childhood

Any sensitive parent or researcher is well aware of the tremendous importance of the first few years of a child's life to his future development and well-being. The parent considers the needs of the child as well as the expectations of society and then attempts to mold and shape this crying, struggling organism into a self-sustaining functional member of society. Each culture in its wisdom promotes those behaviors and values that are easiest to establish biologically. Until recently, the biological guidelines were clearcut. A parent would observe the sex of the newborn infant and proceed to teach the child in accord with the prescribed cultural pattern. However, changes in cultural necessities have alerted the sensitive parent to reexamine the needs of the individual child in light of the latest medical and psychological knowledge.     

Systematic scaling up of neonatal care in countries

Every year about 70% of neonatal deaths (almost 3 million) happen because effective yet simple interventions do not reach those most in need. Coverage of interventions is low, progress in scaling up is slow, and inequity is high, especially for skilled clinical interventions. Situations vary between and within countries, and there is no single solution to saving lives of newborn babies. To scale up neonatal care, two interlinked processes are required: a systematic, data-driven decision-making process, and a participatory, rights-based policy process. The first step is to assess the situation and create a policy environment conducive to neonatal health. The next step is to achieve optimum care of newborn infants within health system constraints; in the absence of strong clinical services, programmes can start with family and community care and outreach services. Addressing missed opportunities within the limitations of health systems, and integrating care of newborn children into existing programmes--eg, safe motherhood and integrated management of child survival initiatives--reduces deaths at a low marginal cost. Scaling up of clinical care is a challenge but necessary if maximum effect and equity are to be achieved in neonatal health, and maternal deaths are to be reduced. This step involves systematically strengthening supply of, and demand for, services. Such a phased programmatic implementation builds momentum by reaching achievable targets early on, while building stronger health systems over the longer term. Purposeful orientation towards the poor is vital. Monitoring progress and effect is essential to refining strategies. National aims to reduce neonatal deaths should be set, and interventions incorporated into national plans and existing programmes.     

Growth of the Pulmonary Valve Annulus after the Modified Blalock-Taussig Shunt in Patients with Tetralogy of Fallot

Background: The surgical outcomes of tetralogy of Fallot (TOF) have evolved dramatically and have resulted in lower mortality rate. Currently, the many cardiac centers have a trend to early single-stage complete repair more than a staged repair. However, the patients who have an early primary repair were required transannular patch augmentation of a pulmonary valve frequently. This effect has been developed a chronic pulmonary insufficiency may lead to right ventricular dilation, dysfunction. In this era, the aim of treatment of TOF is attempted to preserve pulmonary valve annulus for prevent right ventricular dysfunction in the future. The systemic to pulmonary artery shunt is a palliative procedure or known as staged repair for symptomatic patients with TOF. The modified Blalock-Taussig shunt (mBTS) is the most useful systemic to pulmonary shunt and perform as an initial procedure before complete repair. The mBTS can provide increase pulmonary blood flow as well as improve oxygenation and also promote pulmonary artery (PA) growth. However, the effect of this procedure to promote growth of a pulmonary valve annulus is still debate. Objectives: To compare a growth of pulmonary valve annulus between after staged repair and primary repair in patients with TOF (without pulmonary atresia). Methods: A retrospective case-control study, review of patients with TOF underwent total repair at our hospitals from January 2005 and December 2017 was performed, a total number of 112 patients underwent TOF repair. Twenty-nine patients (26%) underwent a staged repair (mBTS group) and 83 (74%) underwent total repair only or primary repair (PR group). We evaluated diameter of pulmonary valve annulus by using echocardiography at the time of first diagnosis and before complete repair on both groups. Results: The age of diagnosis of mBTS group were younger than PR group (p = 0.011). Therefore, pulmonary valve annuls were smaller in mBTS group. (Z-score, −2.93 ± 1.42 vs. −1.89 ± 0.97; p = 0.001). However, the growth potential of pulmonary valve annulus was increase more than PR group significantly (Z-score, −1.46 ± 1.02 vs. −2.11 ± 1.19; p = 0.009) Even though a patent ductus arteriosus was found commonly in PR group (p = 0.018). Conclusions: Our results suggest the systemic to pulmonary shunt or mBTS can promote growth of pulmonary valve annulus in patients with TOF.     

Quality of life of nursing home residents in mainland China: The role of children and family support

Background and objectivesRecently China ended its one-child policy and all married couples were permitted to have two children. The objectives were to investigate: 1) whether having more children (3+ vs. ≤ 2) is positively associated with quality of life (QOL); and 2) the role of child visit frequency and family support in the relationship between number of children and QOL among nursing home residents in mainland China.Research design and methodsThis is a cross-sectional study. A total 371 residents from 33 nursing homes in Jinan, Shandong Province who completed the survey from May to September 2016 were analyzed. The mixed-effects multivariate linear or ordered logistic regression models were used to test the mediating effect of child visit frequency and family support on the relationship between the number of children and QOL. All statistical models were adjusted for resident- and facility-level variables.ResultsNursing home residents with 3+ children received more frequent child visits and family support, and had better QOL compared with those with ≤2 children. However, the relationship between number of children and QOL was attenuated and no longer significant with the inclusion of child visit frequency and family support in the model.Discussion and implicationsThe findings emphasized the importance of the role of children for the QOL of the older adults in a traditionally family-care oriented society like China. As China’s population continues to age, having two children is necessary but may be insufficient in the context of improving nursing home residents’ QOL.     

Human immunodeficiency virus-associated disorders in Pakistan

We have documented evidence of the human immunodeficiency virus (HIV) infection in four individuals including a blood donor and three members of a family (husband, wife, and a child) in Karachi, Pakistan. Our data indicate that HIV has been transmitted to the wife of the seropositive male, a drug abuser, in the recent past and that she has passed the virus to her newborn child perinatally/transplacentally. The two seropositive males (blood donor and drug abuser) were diagnosed clinically as having persistent generalized lymphadenopathy, an AIDS-related condition (ARC), and the child presented with developmental disorders and neurologic manifestations. This is the first report of AIDS/ARC cases from Pakistan that can be directly related to HIV infection and its heterosexual transmission.     

Detection of left ventricular asynchrony in patients with right bundle branch block after repair of tetralogy of Fallot using tissue-Doppler imaging-derived strain

OBJECTIVES: We aimed to investigate whether patients after tetralogy of Fallot (TOF) repair with right bundle branch block have left ventricular (LV) asynchrony and to assess the influence of ventricular asynchrony on regional and global LV function. BACKGROUND: Patients after TOF repair usually have right bundle branch block. However, no data regarding LV asynchrony in this group are available. METHODS: Twenty-five patients after TOF repair and 25 age-matched healthy control subjects were studied. The regional myocardial deformation of the interventricular septum (IVS) and the LV lateral wall were examined using tissue-Doppler-derived strain. The time interval between the onset of QRS complex and the peak strain was measured for each wall. According to the difference between LV and septum time intervals among the normal subjects, a normal range (mean +/- 2 SD) was plotted, and TOF patients in whom the difference was beyond the normal range were considered to have LV asynchrony. The Tei index was used to assess global LV function. RESULTS: Thirteen (52%) of the examined patients after TOF repair had LV asynchrony. Patients after TOF repair with LV asynchrony had a significantly reduced regional septal systolic strain (p < 0.001) and significantly elevated Tei index (p < 0.001) compared with those without. CONCLUSIONS: Left ventricular asynchrony may exist in patients after TOF repair with right bundle branch block. This LV asynchrony is associated with a reduction of both regional and global LV function.     

Prevention of Experimental Allergic Encephalomyelitis with Cobra Venom Factor

Any sensitive parent or researcher is well aware of the tremendous importance of the first few years of a child's life to his future development and well-being. The parent considers the needs of the child as well as the expectations of society and then attempts to mold and shape this crying, struggling organism into a self-sustaining functional member of society. Each culture in its wisdom promotes those behaviors and values that are easiest to establish biologically. Until recently, the biological guidelines were clearcut. A parent would observe the sex of the newborn infant and proceed to teach the child in accord with the prescribed cultural pattern. However, changes in cultural necessities have alerted the sensitive parent to reexamine the needs of the individual child in light of the latest medical and psychological knowledge.