Clinical Manifestation: -

1. Cyanotic episodes or Tet spells: cyanotic spells may occur while crying and after feeding. After cyanotic spells, there may be limpness, fatigue and fainting. The classic description is of a patient who becomes cyanotic and then assumes a squatting position to relieve the cyanosis and hypoxia. Squatting serves to increase peripheral vascular resistance, thereby increasing the pressure in the left heart, and subsequently forcing blood back into the pulmonary circulation.

2. Dyspnoea

3. Delayed physical growth and development

4. Pansystolic murmur may be heard at the middle to lower sternal borders

5. Cyanosis- may be seen mucous membrane of the lips, mouth and pharynx and in fingernails and toe- nails.

6. Clubbing of the fingers

7. Paroxysmal dyspneic attacks (anoxia, “ blue “ spells) occur during the first 24 months of life and last for a few minutes to hours.

Diagnosis:

Cardiac exam: Most importantly, the heart murmur heart in TOF is not due to the VSD! It is infact due to the right ventricular outflow tract obstruction. The murmur is typically crescendo decrescendo with a harsh systolic ejection quality; it is appreciated best along the left mid to upper sternal border with radiation posteriorly. (Remember, an isolated VSD murmur is a holosystolic murmur, best heard in the tricuspid area. It may radiate to the right lower sterna border.) Patients will have a normal S1 and possibly a single S2 due to diminished P2 component.

Blood Studies Show:

Polcythemia and high haemotocrit.

Chest X‐Ray:

As seen on the chest x‐ray below, patients with TOF have right ventricular hypertrophy,a “boot shaped” heart and decreased pulmonary vascular markings.

Electrocardiogram:

On EKG, patients with TOF will show increased right ventricular forces as evidenced by tall R waves in V1. Additionally, right atrial enlargement is manifested by prominent P waves in V1 (*). Right ventricular hypertrophy is demonstrated by a rightward deviated axis.

Echo- evidence of the aortic override, thick anterior right ventricular wall and large aorta.

Treatment:

Once TOF is diagnosed, almost all patients undergo corrective surgical repair within the first year of life. In the interim period, prostaglandin treatment may be necessary to maintain the patency of the ductus arteriosus. Additionally, some patients may require digoxin or diuretics if signs of heart failure are present. Treatment of hypercyanotic spells is directed towards improving pulmonary blood flow. These include oxygen, knee/chest position, morphine, intravenous fluids, sodium bicarbonate, betablockers or pharmacologically increasing systemic vascular resistance by administration of drugs, such as phenylephrine. Once an infant has developed progressive cyanosis or has evidence of hypercyanotic spells, surgical correction is indicated. There are two common surgical procedures:

Palliative Shunts:

1. In infants who cannot undergo primary repair, a palliative procedure to increase pulmonary blood flow and increase oxygen saturation may be performed.

2. A side to side anatomosis of the ascending aorta and right pulmonary artery on neonates (waterson shunt)

3. The most commonly used Blalock taugsig’s procedure for older infants and children in which an artificial ductus is created by anastomosis of a branch of the aorta. (Subclavin artery) to the pulmonary artery.

4. Anastomosis of the upper descending aorta and left pulmonary artery- (pott’s procedure)

Corrective procedure:

Intracardiac repair for TOF:

The ventricular septal defect is closed with a patch. The right ventricular outflow tract is enlarged by opening the RVOT and pulmonary valve, resecting the subinfundibular muscle bundles, and patching the area open. In some cases, a conduit may be inserted to further open the RVOT.

Case Summary:

5 year old child H/O/ CCHD/reduced QP/TOF/SA VSD/INFUNDIFULAR PS/SINGLE S/A VSD and B/L NORMAL LV FUNCTION came to hospital with complaints of cyanotic spell since 2 yrs and now associated with tachypnea, clubbing of the finger, severe cyanotic spells than child admitted in cardiac unit vital signs are taken Heart Rate-98 b/mts, Respiratory Rate- 42 b/mts, BP -102 /54 mm hg SP02-64% ,S1 S2 sounds present and murmur sound also present . Blood investigation done urea-15, creatinine- 0.3, na-142 k- 4.2 ,Ca- 8.4 Uric Acid-.3.3 ,Bilirubin-0.5, SGOT/SGPT- 68/61 Alkaline phosphate-112, HB-10.7 RBC-5.80 HCT – 48.4 , TLC – 16.4, PLATLET- 259, ESR- 22 .Child received treatment inj.cefotaxime 250mg iv tds , tab ciplar 10 mg tds syp: vitcofol 2.5 mg bd than patient posted for surgery .

After surgery ,this patient received with TOTAL CORRECTION (TRANS RA DACRON PATCH VSD CLOSURE + INFUNNDIBULAR RESECTION OF PULMONARY STENOSIS) on PRVC FIO2 60% RR- 18 mts/ min ABG analysis done- PH- 7.31,po2- 275, pco2-38.7,Be- -6.6, HCO3-17.6, Na-140.,K-3.33,ca- 1.0, Hb-12.5 Hct -37.5, Lac-8.6, Cl-105 , vital signs are Bp-70/52 MMHG ,Hr-122 b/mts, RA-4peripheral temperature-27.3,core temperature.39.7 c , child received inotrops support inj: NTG 20/50 @0.6 ml/hr, inj DOPA 200/50@1.0 ML/HR, inj DOBUTAMINE 250/50@ 1.0 ml/hr,ADR 2/50@ 1.3mil/hr, NOR-ADR 2/50@ 1.2 mil/hr .child received treatment inj; Cefotaxime 500 mg iv tds, inj Amikacin 75 mg iv od, inj; pantocid 20 mg iv od inj tremadol 25 mg iv tds, inj perfalgan 250 mg iv qid, ivf N/5 in D5 @ 30 ml/hr. I/O chart maintained With favorable urine output. The patient regained clear consciousness after 12 hours, acceptable arterial blood pressure (80-100/50-70 mmHg) and favorable findings in chest X-ray the patient weaned off from ventilator after 72 hours. after 2 weeks patient discharged successfully

NURSING MANAGEMENT:

1) Decreased cardiac output related to decreased myocardial contractility and reduced stroke volume, as evidenced by: decreased peripheral pulses,peripheral temperature and increased core temperature .

Expected outcome:

To improve the cardiac output

Nursing intervention:

Ø Checked the peripheral circulation (e.g., check peripheral pulses, capillary refill, color, and extremity temperature). Peripheral temperature is low 27 C

Ø Checked the core temperature (39.8c) it indicate low cardiac output than cold sponge, cold saline lavage and tepid sponge given.

Ø Evaluated RA, pulmonary artery and systemic pressures,

Ø Monitored sensory and cognitive capacities, child is conscious.

Ø Administered positive inotropic/contractility medications such as adrenaline , nor adrenaline, dopamine ,dobutamine according to physician order

Ø Monitoring dysarrythmias, urine output, intake output hourly and then checked serum electrolytes especially sodium, potassium, calcium BE, HCO₃, Lactate

Ø Maintained fluid balance by administering IV fluids or diuretics

Ø Monitored and documented heart rate, rhythm, and pulses

Ø Evaluated effects of fluid therapy

Evaluation:

Ø Patient saturation was maintained

Ø Pulse and BP maintained normal

Ø Patient perfusion status was improved

2) Nursing diagnosis:

Ineffective airway clearance related to thick sputum secondary to ventilatorsupport as evidenced by increase airway pressure, rapid respiration, diminished and adventitious breath sounds, and thick white sputum secretion

Expected outcome:

To improve the airway pattern

Nursing intervention:

· Monitoring rate, rhythm, depth, and effort of respirations

· Monitoring chest x-ray reports

· Routinely monitoring ventilator settings

· Checked all ventilator connections regularly

· Monitoring for adverse effects of mechanical ventilation: infection, barotrauma, reduced cardiac output

· Monitoring patient’s respiratory secretions

· suctioning done with hyproxygenation and asthalin nebulization by auscultating for crackles and bronchi over major airways

· Monitoring that ventilator alarms are on or off

· Monitoring for decrease in exhaled volume and increase in inspiratory pressure

· Provided routine oral care in every shift.

· Checked the change in sp02, tidal volume and change in arterial blood gas level.

Evaluation:

Saturation was 98%, every 4^th hourly patient airway cleared by performing suctioning, respiratory secretion color, quantity was assessed, ABG values shows normal.

3) Nursing diagnosis:

Deficit fluid volume related cardiac surgery as evidenced by reduced RA pressure, low BP, weak pulses and poor capillary refill

Expected outcome:

To improve the fluid and electrolyte balance

Nursing Intervention:

· Assessed for signs and symptoms of fluid and electrolyte deficit like decreased RA pressure, low BP, hypokalemia, hyponaterimia and hypocalcimia symptoms

· Monitored vital signs and CVP

· Monitored fluid intake hourly

· Checked the urinary output , chest tube drainage hourly

· Maintained chest tube patency and care given to prevent infection .

· Monitored intake and output chart every hourly .

· Monitored laboratory values like NA, K, Ca, and arterial blood gas 3^rd hourly. Replaced the electrolytes losses ,as indicated

· Maintained a supine or trendenberg position during hypovolemia.

· Administer the IV solutionN/5 in D5 @ 30 ml/hr as child need

Evaluation:

Patient Intake and output chart well balance , Pulse and BP normal

4) Nursing diagnosis:

Impaired physical mobility related presence of ventilator support , intercostal drainage, and ICU stay

Expected outcome:

To improve the physical mobility

Nursing intervention:

· Back care and massage given to prevent friction and improve the circulation

· Patient position changed every 2 hours as evidence by nursing documentation

· Applied air matterss to prevent skin pelling and pressure sore

· Air filled gloves kept under heels.

· Applied soft pad(alwyn pad) under bony prominence area like shoulder blade, sacrum and occipit

· Kept some soft pad under ear cartilage .

· Pt wound (chest and intercostal drainage dressing ) changed daily per wound care order

· proper hand hygiene e performed before and after dressing changes

· Performed Range of Motion exercise every 2^nd hourly in order to maintain the mobility .

· Applied anti emboli stocking to prevent DVT

Evaluation:

Patient mobility, muscle strength maintained effectively and absence of DVT indicate the mobility pattern maintained

5) Nursing Diagnosis :

Risk for infection evidenced by inadequate primary defenses (immature immune system,and altered peristalsis), increased environmental exposure to pathogens, multiple invasive procedures

Expected Outcome:

To reduce the infection

Nursing Intervention:

· Do the observance of universal precautions

· Followed appropriate wound care technique

· Provided the chest tube insertion site care to prevent infection .

· Monitored for systemic and localized signs and symptoms of infection

· Monitor Temperature, signs of infection .

· Inspected condition of any surgical incision/wound

· Ensured aseptic handling of all IV lines Obtain cultures, as needed

· Monitored absolute granulocyte count, WBC count and a differential results CRP, PROCALCITONIN

Evaluation: Patient WBC count was normal, All the universal precaution and patient there was no sign of infection.

6) Nursing Diagnosis:

Acute pain (Chest) related to cardiac surgery or invasive and non invasive painful procedure as evidenced by fatigue, restlessness

Expected outcome:

To reduce the pain

Nursing intervention:

· Assessed the pain which include location,duration, frequency, quality, and severity of pain characteristic, the child behavior pattern during hospitalization.

· Assessed the vital signs, change in behaviours, change in sleep

· Provided supportive care by rest, change of position

· Encouraged the child to express the emotion

· Administered the pain medication as per physician order

· Explore the use on non-pharmacologic pain control measures.

Evaluation:

Pain score was reduced, BP, Pulses, shows normal.

7) Nursing Diagnosis:

Fear and anxiety related to prolonged ICU stay

Expected outcome:

To reduce the child anxiety level.

· Assessed the child behavior pattern during hospitalization

· Encourage to express felling and concerns about illness and hospitalization.

· Familiarize patient with the environment and new experience

· Interact with child in peacefully manner

· Converse using a simple language and brief statement

· Avoid unnecessary reassurance this may increase undue worry

· Encouraging child to write or ask the questions and to discuss with the health team members

Evaluation: Patient doubts clarified

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Received on 14.06.2017 Modified on 15.07.2017

Int. J. of Advances in Nur. Management. 2018; 6(1): 26-30.

DOI: 10.5958/2454-2652.2018.00006.9