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Total anomalous pulmonary venous connection
Patricia SteinTotal anomalous pulmonary venous connection (TAPVC) is a congenital heart defect that occurs in 1.5% of infants with cardiovascular malformations. (1) In TAPVC, there is no connection between the pulmonary veins and the left atrium. Rather, the pulmonary veins establish an anomalous connection that delivers pulmonary venous blood to the right side of the heart rather than to the left side. The right atrium, therefore, receives not only the entire systemic venous return as it should but also receives the entire pulmonary venous return. As a result, the left atrium has no direct pulmonary venous supply. A patent foramen ovale or an atrial septal defect provides a right-to-left shunt so that an admixture of oxygenated and unoxygenated blood eventually reaches the left side of the heart. Without the right-to-left shunting of blood, the infant would die.
Depending on the type of venous connection, TAPVC may be either obstructed or nonobstructed. Immediate surgery is required if the venous connection is obstructed. This article describes
* normal embryology,
* abnormal embryology that results in TAPVC,
* types of anomalous connections,
* how TAPVC is diagnosed,
* surgical correction, and
* nursing Interventions when caring for patients with TAPVC and dealing with the concerns of their family members.
EMBRYOLOGY
The heart, which develops from the cardiogenic mesoderm, is one of the earliest differentiating and functioning organs during embryonic development. Vascular development occurs in many places but most obviously in early formation of the heart. (2)
The heart grows rapidly, creating a cardiac "bulge" seen in the early embryo. It forms initially in the embryonic disc as a simple paired tube inside the forming pericardial cavity. When this disc folds, the heart moves into the correct anatomical position within the chest cavity. (2)
Peripheral vasculature in the developing embryo can be categorized as three distinct aortic and venous circulatory systems:
* the placental (ie, umbilical) system;
* the embryonic system; and
* the vitelline system.
The placental system develops primarily in the extraembryonic mesoderm of the chorion. Its function is to remove embryonic waste and act as a conduit for maternal nutrition. This conduit is not present at birth. The embryonic system begins on both the right and left sides of the embryo and eventually matures into the fully developed cardiovascular system. The vitelline system covers the surface of the yolk sac and contributes to circulation between the gut (ie, embryonic digestive tube consisting of foregut, midgut, and hindgut) and liver. (2)
The lungs develop as outpouchings of the foregut and share the foregut's splanchnic vascular plexus. (3) At this point, there is no direct connection from the lungs to the heart. The paired common cardinal and umbilicovitelline veins connect the plexus to the primitive heart. (3)
At 25 to 27 days of gestation, the developing pulmonary venous plexus retains connections to the right superior vena cava, left superior vena cava, and portal system. No direct communication to the left atrium exists. (2)
At 27 to 29 days of gestation, the primitive pulmonary vein appears as an out-pouching from the left atrium that eventually makes contact with the primitive pulmonary venous system. (2) The right common cardinal system later evolves into the right sinus venosus, which in turn becomes the right superior vena cava and azygos vein. The left common cardinal vein evolves into the left sinus venosus; this becomes the left superior vena cava and coronary sinus. The umbilicovitelline system becomes the inferior vena cava, ductus venosus, and portal vein (Figure 1). (2)
[FIGURE 1 OMITTED]
Connection between the primitive pulmonary vein and pulmonary venous plexus occurs by 30 days of gestation. The common pulmonary vein incorporates into the back wall of the left atrium. Normally, the pulmonary venous portion of the splanchnic plexus gradually loses its connection with the cardinal and umbilicovitelline veins. (2,3)
ABNORMAL EMBRYONIC DEVELOPMENT
With the anomalous connection that occurs in TAPVC, the common pulmonary vein either does not connect with the pulmonary venous system or does not form at all. Right atrial and right ventricular enlargement occur because all pulmonary venous return connects to the systemic venous system. (3) Persistence of the right and left cardinal veins and the umbilicovitelline all create, at one junction or another, a total anomalous pulmonary venous connection. If neither pulmonary nor systemic connection is available to the lungs, then death will occur during the neonatal period (ie, the first 28 days of life). (4)
An atrial septal defect or patent foramen ovale serves a vital function in keeping these patients alive because it is the only way for the left ventricle to receive blood, albeit an admixture of both oxygenated and unoxygenated blood. A ductus arteriosus frequently accompanies this anomaly. (5) Seventy-five percent of patients with TAPVC have a widely patent connecting vein with no obstruction of venous return; (4) the remaining 25%, however, have narrowed and obstructed systemic venous blood flow. (4)
Other cardiac malformations can coexist with TAPVC. Congenital heart disease (CHD) often is accompanied by other cardiac defects. For example, an atrial septal defect can be associated with bilateral superior vena cava, absence of a coronary sinus, an endocardial cushion defect, right ventricular outflow tract obstructions, transposition of the great arteries, and isomerism of the atria and right- or left-sided heart apex. Thus, if CHD is present, it is likely that another cardiac anomaly is present as well. Furthermore, 75% of patients with asplenia have TAPVC. (5)
In the population-based, Baltimore-Washington Infant Study, a stratified, random sample of 522 live-born infants born between 1981 and 1987 was reviewed. (6) The incidence of TAPVC was 1.5%. Noncardiac malformations were present in 22% of those patients. Patients with TAPVC had a lower birth weight, younger gestational age, and intrauterine growth retardation. The study also reported an association between-TAPVC and self-reported maternal lead exposure during the first trimester and an association with paternal exposure to lead six months before conception occurred. (6,7)
TYPES OF ANOMALOUS PULMONARY VENOUS CONNECTIONS
Total anomalous pulmonary venous connection is classified according to the site where the connection occurs--supracardiac, cardiac, infracardiac, or a mixture of two or more types (Figure 2).
* Supracardiac sites involve connections to the right superior vena cava; azygous vein; or more commonly, to the left superior vena cava or left innominate vein. At least 50% of all incidences of TAPVC are supracardiac. (4)
* Cardiac sites involve connections to the coronary sinus or right atrium.
* Infracardiac sites involve connections below the diaphragm to the portal venous system; ductus venosus; or more rarely, the inferior vena cava.
* Mixed-type sites can be a combination of any of the above connections. The condition is further categorized as either obstructed or nonobstructed. When it is obstructed, immediate surgery is required. Not all types cause obstruction, but when present, obstructions occur in a variety of ways. For instance, the pulmonary vein may be extrinsically compressed between the left main stem bronchus and left pulmonary artery. This squeezes the vein a vise-like manner (Figure 3), which occurs when pulmonary arterial pressure increases, essentially trapping the compliant vein. (4)
[FIGURES 2-3 OMITTED]
In infracardiac TAPVC, the venous channel passes through the diaphragm via the esophageal hiatus. Each time the infant swallows or the diaphragm contracts, the vein is compressed. (4) If the foramen ovale is narrowed, obstruction occurs as it does in the infradiaphragmatic connection. When the anomalous connection is to the portal vein, the high resistance of the hepatic parenchymal circulation creates its own barrier to blood flow for the pulmonary vein. (4)
An obstructed pathway creates a host of adaptations by the heart. The right ventricle tends toward hypertrophy, but the right atrium of the heart is not dilated because pulmonary blood flow is limited by the anomalous connection. The left ventricle, lacking in volume and demand, does not supersede the right ventricle in size as it normally would when given the proper circulatory routing for ejection into systemic circulation. (4)
DIAGNOSING TAPVC
The way in which TAPVC is diagnosed depends on whether pulmonary venous obstruction is present. Health care providers use a variety of diagnostic methods to definitively diagnose the presence of TAPVC (eg, clinical examination, chest x-rays, electrocardiogram [ECG], echocardiogram, cardiac catheterization, serial sampling of oxygen saturation levels).
DIAGNOSING TAPVC IN AN INFANT WITH AN OBSTRUCTION, On clinical examination of an infant with TAPVC in whom an obstruction is present, a health care practitioner will note that the infant is critically ill and exhibiting signs and symptoms of marked cyanosis, tachypnea, dyspnea, hypoxemia, and metabolic acidosis. In extreme hypoxia, the infant may develop disseminated intravascular coagulation. (5) A chest x-ray may show a ground-glass appearance. An ECG will demonstrate right ventricular hypertrophy and right-axis deviation. (4) An echocardiogram is helpful for tracing the connections of all the pulmonary veins. No connections between the pulmonary veins and left ventricle should be visible in an echocardiogram, and it will show right-to-left shunting indicating that an atrial septal defect is present. (4) Cardiac catheterization is helpful in identifying the exact anatomy of the pulmonary venous system, its degree of obstruction, pulmonary hypertension, and any other structural abnormalities. In preparation for surgery, the results of the catheterization will help health care practitioners anticipate the size of the atrial septal defect. Serial sampling of oxygen saturation levels throughout the venous system also help localize the specific site of the anomalous connection. (4)
DIAGNOSING TAPVC IN AN INFANT WITHOUT AN OBSTRUCTION. A health care practitioner will note mild cyanosis and congestive heart failure during clinical examination of a patient with TAPVC that is not obstructed. The infant will experience frequent respiratory tract infections and grow more slowly than infants in the same age group. The infant would demonstrate evidence of cardiomegaly that eventually affects lung expansion and ventricular function. The infant's ECG will show right ventricular enlargement and the echocardiogram will show a dilated right ventricle. (4)
SURGICAL TREATMENT
If a patient who has TAPVC also has an obstruction, it is considered a surgical emergency. Immediate mortality after TAPVC repair ranges from 2% to 20%. Depending on the series of patients studied, success rate ranges from 80% to 98% for repair of TAPVC; the success hinges on the degree of pulmonary venous obstruction. (4,5) At least 5% to 10% of patients experience a late failure as a result of pulmonary venous obstruction, (3) although the cure rate can be as high as 90%. (3)
The postoperative course is affected by the anesthetic agents used, amount of time on cardiopulmonary bypass (CPB), aortic cross-clamp time, depth of hypothermia, and duration of circulatory arrest. (8) Bypass alone affects coagulation, platelet function, electrolyte balance, glycemic control, and extravascular fluid accumulation, which affects the length of time the patient will have to remain in the neonatal intensive care unit (NICU). (8) In an emergent situation, the immediate surgical objective is to
* connect the common pulmonary venous channel to the left atrium,
* divide the vertical pulmonary vein, and
* close inter-arterial shunts, if present.
The surgery demands much of a perioperative nurse in terms of organization, planning, and execution. He or she must be well prepared to begin the TAPVC procedure by ensuring that all necessary equipment is available in the OR suite and functioning properly. The perioperative nurse ensures that the defibrillator is in the room, functioning correctly, and ready to be connected to the external and internal paddles. He or she ensures that warming devices for blankets and fluids are in the room. The circulating nurse positions the OR bed and drops the foot to allow for the bed to more closely match the size of the patient. The nurse increases the room temperature and places a forced-air, temperature-regulating blanket on the bed to help maintain the patient's core body temperature before and after CPB. The nurse ensures that headlights and optical loops are available for the surgeon for positioning suture and identifying small bleeding vessels.
The patient's condition may require initiating CPB sooner rather than later. Consequently, the circulating nurse and scrub nurse ensure the availability of appropriately sized cannulas and required suture. Many laboratory specimens will be drawn to assess how well the patient is tolerating surgery. The nurse ensures the availability of arterial blood gas and blood testing supplies and forms for blood gas analyses and electrolyte testing during surgery. The circulating nurse, scrub person, and anesthesia care provider ensure that intake and output are strictly monitored throughout the intraoperative phase.
In all situations, not just emergencies, the arterial duct is identified and doubly ligated immediately after CPB is established. (3) The inter-arterial shunt must be ligated before repair to prevent air from entering the systemic circulation, which could cause cerebral damage. (3) Both a single venous and single arterial cannula are used for all forms of TAPVC. Following are the general steps for surgical repair of the main types of TAPVC (ie, supracardiac, cardiac, infracardiac).
SUPRACARDIAC TAPVC. The surgeon ligates the left-sided vertical vein at its junction with the innominate vein. He or she then opens the left atrium and incises the posterior wall. The surgeon then incises the anterior wall with the venous confluence. He or she creates an anastomosis of the pulmonary venous confluence to the left atrium and closes the atrial septal defect or patent foramen ovale (Figure 4). When the connection is to the azygous vein, the surgeon ligates and performs a direct anastomosis of the venous confluence to the posterior aspect of the left atrium. (3)
[FIGURE 4 OMITTED]
CARDIAC CONNECTION TO THE CORONARY SINUS. The surgeon makes a large, inter-atrial connection by incising the septum of the coronary sinus and septum primum. With the separated left and right atria, the surgeon closes the newly created atrial septal defect and coronary sinus ostium using a patch. (3) The defect remaining in the coronary sinus allows all pulmonary venous and coronary sinus return to drain into the left atrium (Figure 5).
[FIGURE 5 OMITTED]
CARDIAC CONNECTION TO THE RIGHT ATRIUM. The surgeon partially excises the atrial septum and sews a patch to the edges of the defect and posterior right atrial wall. This causes pulmonary venous blood to flow directly through the defect into the left atrium (Figure 6).
[FIGURE 6 OMITTED]
INFRACARDIAC CONNECTION, After establishing CPB, the surgeon ligates the descending vertical vein (ie, a vein left over from the umbilical system that is part of the original anomalous pathway) at the level where it enters the diaphragmatic hiatus. He or she opens the left atrium and incises the posterior wall. The surgeon also incises the anterior wall of the venous confluence, which he or she anastomoses to the left atrium, and then closes the patent ductus with a patch (Figure 7). (3)
[FIGURE 7 OMITTED]
PERIOPERATIVE NURSING INTERVENTIONS
It is imperative that the perioperative nurse perform a preoperative assessment to obtain details about the patient's cardiac history. The infant may be cyanotic as evidenced by a slate-blue color of the mucous membranes, nails, and skin. (9) Altered renal function from decreased perfusion of the kidneys will affect urine output. The patient may have weak distal pulses, tachycardia, tachypnea, and skin mottling. (9) The perioperative nurse should develop and initiate a nursing care plan with nursing diagnoses appropriate to caring for a patient undergoing surgical repair of TAPVC based on this assessment (Table 1). The nursing care plan should incorporate the infant's family members, who will be waiting anxiously for their infant to come safely out of the OR. The nurse should consider the following nursing diagnoses for the family and infant:
* potential for injury to include
** risk for altered thermoregulation,
** risk for impaired skin integrity, and
** risk of infection related to surgical procedure and
* communication issues to include
** anticipatory grieving versus anxiety and fear related to pending surgery and associated with the potential loss of their child,
** knowledge deficit, and
** risk for altered family coping and parent-infant bonding.
POTENTIAL FOR INJURY
The potential for injury to the patient exists before, during, and after surgical repair of TAPVC. A one-day-old patient for whom the surgical procedure is an emergency is gravely ill. When placing an indwelling urinary catheter, the nurse should remember that the urethra is very short in both male and female infants. When catheterizing male infants, the nurse should avoid injuring the infant by taking care not to inflate the balloon until the catheter is in the bladder.
All surgical team members should watch for anything that may crush the patient's limbs, toes, or fingers. The infant's head and limbs will likely be limp and flaccid before surgery and even more so during and after anesthesia. Particularly, infants' heads are oversized and are difficult to control if not properly supported. Some patients may come to the OR already intubated, so surgical team members should take special care to keep these patients well supported when transferring them to and from the NICU and onto and off of the OR bed to ensure that all invasive lines and tubes remain intact.
Desai et al (10) stressed the importance of trying to minimize external stimuli; it is possible to regulate lighting and noise in the OR suite itself. They also suggested swaddling. This is not possible intraoperatively but may be possible to a limited extent postoperatively before the patient is transferred to the NICU.
RISK FOR ALTERED THERMOREGULATION. Hypothermia is a concern for any infant undergoing surgery (11) and is particularly a risk for infants with TAPVC. Infants are less able than adults to regulate their temperature, especially when undergoing general anesthesia, because of the large skin-to-surface area compared to body mass. (11) Consequently, warming is vital, but particular attention needs to be taken to prevent burns from over warming or from the blanket coming into direct contact with the infant's skin.
Despite intended cooling during CPB, measures must be taken to keep the infant warm before and after CPB. The circulating nurse should loosely wrap the infant's arms and legs with soft, non-binding material. The nurse also should apply warming blankets below the infant's umbilicus and cover the infant's head with a plastic cover. The nurse also can encircle the patient with a forced-air, temperature-regulating blanket specific for pediatric patients. Near the end of the procedure, the circulating nurse should ensure that the infant's crib is warmed with forced warm air in anticipation of transfer to the NICU.
RISK FOR IMPAIRED SKIN INTEGRITY. The perioperative nurse should perform a very thorough skin assessment. There is a high likelihood of prolonged immobility and intubation before, during, and after surgery. Poor perfusion preoperatively means decreased capillary filling, which also puts the patient at risk for skin breakdown. When prepping, the circulating nurse should ensure that no pooling of prep solution occurs. Typically, infants are grounded on their backs, so the circulating nurse should not allow prep solution to drip down the patient's side and interrupt contact with the electro-surgical unit dispersive pad.
RISK OF INFECTION RELATED TO THE SURGICAL PROCEDURE. If surgery has not occurred on an emergent basis, the patient is likely to have sustained several respiratory infections during his or her short lifetime before surgery. (4) Furthermore, the patient may have signs of congestive heart failure. (4) Impaired circulation of purely oxygenated blood affects all aspects of the patient's preoperative condition going into surgery. An optimal time to perform the surgical procedure may be between recurring infections. The entire surgical team should be aware of the critical importance of adhering to aseptic technique for these at-risk patients.
COMMUNICATION ISSUES
One pivotal role of the perioperative nurse during cardiac surgery is to be the conduit for clear, concise, and consistent communication to the parents about what will happen and how their infant will be cared for and to provide needed updates throughout the procedure. This requires careful and sensitive delivery of information as well as active listening to the family members' concerns.
To parents, a diagnosis of a congenital heart defect is a life-or-death situation. According to Pye and Green, (12)
The moment an infant is diagnosed with a congenital heart defect, parents experience a mixture of shock, disbelief, fear, anger, and often a profound sense of sadness.
Desai et al (10) called this time period a life-changing experience, even if the parents knew prenatally that their child would be born with a congenital heart defect. This life-changing experience is compounded when the newborn infant, sometimes only one day old, is taken to surgery to undergo a major, lifesaving surgical procedure. Sometimes, entire families and extended families will hold vigils outside the OR during surgery and again when the infant is out of surgery.
ANTICIPATORY GRIEVING VERSUS ANXIETY AND FEAR RELATED TO PENDING SURGERY, The very real fear that the surgical procedure may not help is palpable for the family members of infants with TAPVC. Family members may begin a grieving process associated with the potential loss of their child if surgical therapy is ineffective. Congenital heart defects are often accompanied by other defects. (13,14) Thus, a "return to normal" is a relative term. The TAPVC surgical repair can be successful, but the final patient outcome is interlinked with any other defects that the patient also has, which will affect the long-term prognosis. The perioperative nurse must understand the intensity of these feelings and help the family cope with them. The circulating nurse can provide family members with periodic updates to help alleviate their fears. For family members, not hearing any word from the OR, even if things are going fine, is much more stressful than receiving a call that checks in with them during surgery. Many nurses make it a habit to call the family when the patient begins CPB just to give them a sense of the progress of the procedure.
KNOWLEDGE DEFICIT. The high stress of surgery may prevent the parents from fully understanding exactly what will be happening to their infant. The recovery period also is an unknown and can be overwhelming.
Postoperatively, the patient goes directly to the NICU. The sight of the infant after surgery can be very frightening for the parents and family members. The many tubes, multiple IV drips, noises, and monitors
may lead the family members to believe that the patient is not better after surgery but is, in fact, worse. Thus, the perioperative nurse should inform family members before surgery about what they are likely to see when they first observe their infant after surgery. Although it may produce anxiety, information can give family members a sense of control and thus, help to allay some fear. Postoperative teaching involves a multidisciplinary approach because of the variety of issues with which the family members must deal. (10)
In many centers around the country, a child-life specialist will be assigned to the family to help them cope with the stress of this diagnosis. The child-life specialist provides help in a variety of ways, such as escorting the family members to the surgical waiting room and discussing their fears associated with the surgical procedure. (10)
RISK FOR ALTERED FAMILY COPING AND PARENT/ INFANT BONDING, Many things affect the amount energy that parents may have to take in new information, such as having another child at home, which inevitably divides the parents' attention. (10) An ongoing relationship with the team of health care providers can be very helpful for the family as questions arise. Parents can be nervous about mortally harming their infant. Reassurance and close follow-up will help with these concerns and ensure appropriate bonding between the infant and his or her parents as well as between the infant and other family members, such as siblings and grandparents.
CHALLENGES OF CARDIAC SURGERY FOR PEDIATRIC PATIENTS
Open-heart surgery for any patient is a challenge for the surgical team, the patient, and the family. Pediatric patients present an even greater challenge. A multidisciplinary team approach of caring for family members emphasizes the message that their infant is the team's first priority. (10)
Although surgical repair of TAPVC is a fairly straightforward procedure, these patients may be quite ill and require intensive support before, during, and after surgery. In particular, one-day-old patients requiring immediate surgery are fragile. Family members also are fragile and must be cared for with the same attention and concern as the patient. It is vital that perioperative nurses caring for these tiny patients understand the heightened attention to detail required to contribute to the success of the surgery.
Examination
Total Anomalous Pulmonary Venous Connection
PURPOSE/GOAL
To educate perioperative nurses about caring for infants undergoing surgical repair of total anomalous pulmonary venous connection.
BEHAVIORAL 0BJECTIVES
After reading and studying the article on total anomalous pulmonary venous connection (TAPVC), nurses will be able to
1. describe TAPVC,
2. compare normal and abnormal embryologic development of the cardiovascular system,
3. describe how types of anomalous pulmonary connections cause the signs and symptoms of TAPVC,
4. describe surgical treatment of infants with TAPVC, and
5. discuss perioperative nursing care of patients undergoing surgical repair of TAPVC.
QUESTIONS
1. Total anomalous pulmonary venous connection (TAPVC) is a congenital heart defect in which
1. there is no connection between the pulmonary veins and the left atrium.
2. the pulmonary veins deliver pulmonary venous blood to the right side of the heart.
3. the left atrium receives systemic venous return and the right atrium receives pulmonary venous return.
4. the left atrium has no direct pulmonary venous supply.
a. 1 and 3
b. 2, 3, and 4
c. 1, 2, and 4
d. 1, 2, 3, and 4
2. The fully developed cardiovascular system evolves from the
a. embryonic system.
b. placental (ie, umbitical) system.
c. vitelline system.
3. With TAPVC, the only way for the left ventricle to receive blood before surgical repair is by
a. the presence of a patent foramen ovale or an atrial septal defect.
b. cardiopulmonary bypass.
c. an intra-aortic balloon pump.
4. Total anomalous pulmonary venous connection is classified according to
a. the location of the obstruction.
b. the site where the anomalous connection occurs.
c. the severity of the infant's congestive heart failure.
5. On clinical examination of an infant with TAPVC in whom an obstruction is present, a health care practitioner will note that the infant is critically ill and exhibiting symptoms of marked
1. cyanosis.
2. dyspnea.
3. hypoxemia.
4. tachypnea.
a. 1 and 3
b. 2 and 4
c. 1, 2, and 4
d. 1, 2, 3, and 4
6. Success for repair of TAPVC hinges on the degree of pulmonary venous obstruction.
a. true
b. false
7. The length of time an infant will have to remain in the neonatal intensive care unit is dependent on the effect of cardiopulmonary bypass on
1. coagulation.
2. electrolyte balance.
3. extravascular fluid accumulation.
4. glycemic control.
5. platelet function.
a. 1 and 3
b. 2 and 4
c. 1, 3, and 5
d. 1, 2, 3, 4, and 5
8. In an emergent situation, the immediate surgical objective is to
1. connect the common pulmonary venous channel to the left atrium.
2. divide the vertical pulmonary vein.
3. close inter-arterial shunts, if present.
a. 1 and 2
b. 2 and 3
c. 1, 2, and 3
9. The inter-arterial shunt must be ligated before repair
a. to allow access to the ventricle.
b. to prevent air from entering the systemic circulation.
c. to prevent inadvertent damage of the valve.
10. Appropriate nursing interventions for a patient undergoing surgical repair of TAPVC include
1. implementing thermoregulation measures.
2. evaluating the effectiveness of bonding between the infant and parents.
3. initiating necessary referrals for family support services.
4. ensuring pain management caused by distal ischemia of the extremities.
5. using appropriate communication skills to ease fears and improve comprehension of the patient's family members.
a. 1 and 5
b. 2, 3, and 4
c. 1, 2, 3, and 5
d. 1, 2, 3, 4, and 5
The behavioral objectives and examination for this program were prepared by Rebecca Holm, RN, MSN, CNOR, clinical editor, with consultation from Susan Bakewell, RN, MS, BC, director, Center for Perioperative Education.
The author acknowledges the valuable support and assistance of James Moiler, MD, the Paul Dwan professor of education in pediatric cardiology at the University of Minnesota, Minneapolis.
REFERENCES
(1.) Wilson AD. Total anomalouspulmonary venous connection, eMedicine. Available at: http: //www.emedicine.com/ped/topic2540.htm. Accessed January 25, 2007.
(2.) Hill M. Cardiovascular system development. UNSW Embryology. January 2006. Available at: http://embryology.med.unsw.edu.au/Notes/heart.htm. Accessed January 3, 2007.
(3.) Venoatrial malconnections. PediHeart.org. Available at: http://www.pediheart.org/ practitioners/defects/venoatrial.htm. Accessed January 3, 2007.
(4.) Amplatz K, Moiler J. Radiology of Congenital Heart Disease. St Louis, Mo: Mosby-YearBook; 1993:805-825.
(5.) Total anomalous venous connection and cor triatriatum. Cardiothoracic Surgery Notes. March 10, 1997. Available at: http://www.cts net.org/doc/5387. Accessed January 3, 2007.
(6.) Correa-Villasenor A, Ferencz C, Boughman JA, Neill CA, The Baltimore-Washington Infant Study Group. Total anomalous pulmonary venous return: familial and environmental factors. Teratology. 1991;44:415-428. Also available at: http://www3.inter science.wiley.com/cgi-bin/jissue/1105 13039. Accessed January 3, 2007.
(7.) Jackson LW, Correa-Villasenor A, lees PS, et al. Parental lead exposure and total anomalous pulmonary venous return. Birth Defects Res A Clin Mol Teratol. 2004;70:185-193. Available at: http://www.interscience.wiley.com/cgi-bin/jissue/108069806. Accessed January 3, 2007.
(8.) Merle C. Nursing considerations of the neonate with congenital heart disease. Clin Perinatol. 2001;28:223-233.
(9.) Westmoreland D. Critical congenital cardiac defects in the newborn. J Perinat Neonatal Nurs. 1999;12:67-87.
(10.) Desai P, Ng J, Bryant S. Care of children and families in the CICU: a focus on their developmental, psychosocial and spiritual needs. Critical Care Nurs Q. 2002;25:88-97.
(11.) Leben J, Tryba M, Kurz-Muller K, Schregel W. Prevention of intraoperative hypothermia in children. Anaesthetist. 1998;47:475-478.
(12.) Pye S, Green A. Parent education after newborn congenital heart surgery. Adv Neonatal Care. 2003;3:147-156.
(13.) Jeanty P, Silva SR, Leite J. Aspleniapolysplenia syndromes. January 18, 2006. TheFetus.net. Available at: http://www.thefetus.net/page.php?id=407. Accessed January 3, 2007.
(14.) Jaiyesimi FO, Ruberu DK. Noncardiac defects in children with congenital heart disease. Available at: http://www.kfshrc.edu.sa/annals/143/93010.html. Accessed January 3, 2007.
From Amplatz K, Moller JH. The Radiology of Congenital Heart Disease. St Louis, Mo: Mosby-YearBook; 1993. Reprinted with permission. Illustration by Martin A. Finch.
From Reardon MJ, Cooley DA, Kubrusly L, et al. Total anomalous pulmonary venous return: report of 201 patients treated surgically. Tex Heart Inst J. 1985; 12:131-141. Reprinted with permission. Illustration by Bill Andrews.
Patricia Stein, RN, BSN, MAOL, CNOR, is a nurse education specialist for perioperative services at Palomar Pomerado Health, Escondido, Calif.
TABLE 1
Nursing Care Plan for Patients Undergoing Surgical Repair
of Total Anomalous Pulmonary Venous Connection
Diagnosis Nursing interventions
Risk of * Monitors body temperature throughout the
hypothermia perioperative period.
related to the * Implements thermoregulation measures by
perioperative ** covering the infant's head with a plastic
environment, cover;
patient age, ** wrapping the infant's extremities with
and exposed soft, non-binding material to help mini-
body surfaces. mize skin exposure as much as possible;
** encircling the patient with a forced-air,
temperature-regulating blanket; and
** prewarming the postoperative crib
with forced warm air.
* Evaluates response to thermoregulation
measures.
Risk for * Assesses skin integrity and sensory
infection impairments.
related to * Observes sterile field and perioperative
immature team members to ensure that asepsis is
immune maintained.
system * Validates that preoperative antibiotic was
and poor administered according to facility policy.
nutritional * Allows sufficient time for surgical prep
status. solution to dry before the patient is draped.
Risk for * Elicits family members' perception of
compromised anesthesia and surgery.
family coping * Evaluates effectiveness of bonding
related to between infant and parents.
anticipatory * Evaluates coping support systems avail-
grieving. able to family members.
* Initiates necessary referrals for family
support services.
Risk for known- * Identifies communication barriers and
edge deficit re- knowledge level.
lated to stress * Assesses readiness to learn.
of surgery. * Uses appropriate communication skills to
ease fears and improve comprehension of
the patient's family members, such as
** sitting with family members when per-
forming patient education,
** making eye contact with each parent,
** speaking slowly and clearly and obser-
ving for indications of confusion versus
comprehension, and
allowing time for questions and answers.
* Evaluates family members' response to
instruction.
Interim out- Outcome
Diagnosis come criteria statement
Risk of Patient's core Patient is at
hypothermia body temperature or returning
related to the remains in expect- to normother-
perioperative ed range through- mia at the
environment, out the periopera- conclusion of
patient age, tive experience. the immedi-
and exposed ate postoper-
body surfaces. ative period.
Risk for Patient's wound Patient is
infection is dry, non- free of
related to reddened, and signs and
immature nontender. symptoms of
immune Patient does not health care-
system demonstrate associated
and poor symptoms of in- infection
nutritional fection (eg, wound through the
status. induration, foul 30 days
odor, purulent after the
drainage, fever). perioperative
procedure.
Risk for Parents and Patient's
compromised patient demon- family mem-
family coping strate appropriate bers demon-
related to bonding. strate the
anticipatory ability
grieving. to cope
throughout
the perioper-
ative period.
Risk for known- Patient's family Patient's
edge deficit re- members verbal- family
lated to stress ize understanding members
of surgery. of the procedure, demonstrate
sequence of knowledge
events, and ex- of the
pected outcomes. physiological
and
psychological
responses
to the
procedure.
COPYRIGHT 2007 Association of Operating Room Nurses, Inc.
COPYRIGHT 2008 Gale, Cengage Learning