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Newborns with sig- nificant tricuspid insufficiency pose a particularly difficult surgical challenge buy cheap ditropan 2.5mg online. Patients have undergone varying types of tricuspid valve repairs in the newborn period buy 5 mg ditropan visa, but usually with only limited success buy ditropan 2.5mg low price. Older patients with progressive tri- cuspid insufficiency may benefit from tricuspid valve repair or replacement. Indications for surgery include progressive cyanosis, worsening heart failure, arrhythmias, and paradoxical emboli due to right-to-left atrial shunting. Long-term Follow-up Children with significant tricuspid insufficiency require long-term anticongestive therapy with diuretics and possibly digoxin. However, those patients with mild degrees of tricuspid insufficiency may remain asymptomatic and require no treatment in the early years. It is not uncommon, however, for these patients to develop worsening congestive heart failure or cyanosis due to progressive tricuspid insufficiency during the second or third decade of life. These patients would then need to be treated medically for the heart failure symptoms and surgical repair or replacement of the tricuspid valve should be considered. Patients should be followed closely for the evidence of cyanosis, increasing shortness of breath, increasing fatigue, or for the evidence of arrhythmias. Prognosis The prognosis of Ebstein’s anomaly is directly related to the severity of the valve abnormality and degree of tricuspid insufficiency. It is estimated that the overall mortality rate in the first year of life is around 20%. The average life expectancy for early survivors is 20 years, but there are ample reports of patients with milder forms of Ebstein’s anomaly who live much longer. Cross and Ra-id Abdulla Case Scenarios Case 1 About 6 h after an uncomplicated delivery, it is noted that a full term female infant appears to be cyanotic. Heart examination reveals increased right precordial activity with a right-sided heave. There is a 3/6 systolic regurgitant murmur of tricuspid insufficiency heard along the left lower sternal border and a wide split first heart sound is heard. Chest X-ray demonstrates a markedly enlarged cardiac silhouette and the lung fields are dark, consistent with diminished pulmonary blood flow. An echocardiogram is obtained and shows severe apical displacement of the tricuspid valve into the right ventricle, and there is severe tricuspid valve insuffi- ciency. The right atrium is moderately enlarged and a small atrial septal defect is present. This newborn has severe Ebstein’s anomaly with severe tricuspid valve insuf- ficiency. The right ventricle is unable to produce adequate pressure to overcome the high pulmonary vascular resistance in this newborn. There is also right to left shunting of deoxygenated blood across the atrial septum sec- ondary to the tricuspid insufficiency and high right atrial pressures. The baby needs to be followed over the following days as the pul- monary vascular resistance drops to determine if forward pulmonary blood flow across the small right ventricle improves. The baby can most likely be tried off the prostaglandin E1 in 3–4 days to determine if there is adequate pulmonary blood flow after the pulmonary vascular resistance has decreased. In severe cases, the child may eventually require a univentricular repair (Fontan procedure), however, this is unlikely. His past medical history is unremarkable, although his mother had been told in the past that he had a faint murmur. Chest X-ray demonstrates a mildly enlarged cardiac silhouette, but is otherwise normal. On examination now, his heart rate is 75 bpm, respiratory rate 14 per min, and blood pressure 115/80. Cardiac exam reveals mildly increased right precordial activity, regular rhythm, and normal first and second heart sounds. There is a 2/6 systolic regurgitant murmur at the left lower sternal border and a systolic click is present. His liver edge is palpable 3 cm below the right costal margin, and he is well perfused with 2+ pulses in all extremities. An echocardiogram is obtained and shows moderate tricuspid insufficiency associated with mild apical displacement of the tricuspid valve toward the cardiac apex. The right atrium is also moderately enlarged and the right ventricular function is mildly depressed. This teenager presented with supraventricular tachycardia as a result of Wolff– Parkinson–White type bypass tract associated with mild to moderate Ebstein’s anomaly. He most likely had mild tricuspid insufficiency in the past, but it is now worsened secondary to diminished function due to the supraventricular tachycardia. Immediate treatment could include initiation of diuretics for the treatment of mild heart failure. The heart failure symptoms most likely improve with good arrhythmia control, but he needs to be followed in the future for the progression of tricuspid insufficiency and potential worsening heart failure. Management of Wolff– Parkinson–White syndrome may include medical therapy, but more likely an electrophysiology study with potential ablation of the bypass tract is warranted. Chapter 25 Vascular Rings Ra-id Abdulla Key Facts • Children with respiratory symptoms should be suspected to have vascular ring if: – Symptoms start early in life – Dominant clinical features are stridor and upper airway noises – Children are noted to assume an arched back and extended neck position – Chest X-ray shows evidence of right aortic arch • Double aortic arch present early in life, while right aortic arch with aber- rant left subclavian artery present later in infancy. Definition Vascular ring occurs when the great arteries or their branches assume an abnormal anatomy leading to the formation of a ring of vessels surrounding and constricting the esophagus and trachea. Three types of vascular abnormalities are most common, these are: (1) double aortic arch, (2) right aortic arch with aberrant left subclavian artery, and (3) pulmonary sling. The latter abnormality: pulmonary sling does not form a ring around the esophagus and trachea, but rather a sling around the trachea.

Sports participation should be limited in patients with aortic stenosis based on the degree of the gradient buy ditropan 2.5mg with amex. Mild stenosis (mean gradient <25 mmHg) and normal aortic dimensions have no restrictions on participation 2.5mg ditropan visa. Moderate stenosis (mean gradient between 24 and 40 mmHg) may participate in low intensity competitive sports quality 5mg ditropan. In addition, some athletes may participate in low and moderate static or low and moderate dynamic activities if exercise testing is normal to the level of that activity. Patients who have undergone balloon valvuloplasty, valve repair, or surgical replace- ment also have specific participation guidelines outlined for their conditions. In contrast, patients with acquired valve disease from rheumatic fever or age-related calcification do not have an acceptable response. Asymptomatic patients with peak aortic gradient >60 mmHg or mean gradient >40 mmHg by echocardiographic Doppler. Surgical management is reserved for adults and patients with either aortic stenosis refractory to balloon dilation or those with significant aortic regurgitation. Aortic stenosis may either be managed with valvuloplasty, valve replacement with a Ross procedure (native pulmonary valve moved to the aortic position), or valve replace- ment with a bioprosthetic or mechanical valve. More frequent follow-up is indicated for patients with severe disease, patients who are undergoing rapid growth (first 3–5 years of life and adolescence), athletes, and pregnant individuals. Prognosis Prognosis of aortic stenosis is generally good for patients with mild disease. However, gradients tend to increase with patient age as the aortic valve calcifies, as do the risks of intervention. Most patients who require an intervention in childhood will require additional interventions in adulthood including valve replacement. Females of childbearing age require particular counseling since aortic stenosis increases the risk of pregnancy to both mother and fetus. Furthermore, anticoagula- tion therapy is required following mechanical valve replacement, which is often necessary in adulthood presents significant problems to both mother and fetus because of the teratogenic effects of warfarin and the increased risk of maternal hemorrhage. During a preparticipation sports physical, a previously healthy 14-year old with short stature is noted to have a murmur. McCarville soccer team, he has a brief syncopal episode at the end of the practice. He is responsive quickly upon awakening but is sent to the emergency room for evalua- tion. However, on further questioning, his mother notes that he has had a murmur since 4 years of age when he contracted rheumatic fever. Physical examination reveals a well-appearing, well- nourished African-American male. Heart rate is 80, blood pressure is 125/80, and oxygen saturation is 97% on room air. On cardiac exam, his precordium is mildly hyperdynamic with maximal impulse slightly leftward. There is a systolic ejection click best heard in the fourth intercostal space at the right sternal border followed by a harsh 3/6 ejection murmur with radiation to the neck and apex. There is a short 1/4 diastolic murmur best heard with the patient leaning forward. Extremities are warm and well perfused without the evidence of edema and pulses are 2+ in the right arm and right femoral regions. While this obstruction could be secondary to a number of lesions, including hypertrophic cardiomyopathy, coarctation of the aorta, or sub- or supravalvular aortic stenosis, the click is diagnostic of aortic stenosis. Based on symptoms and physical exam findings in the setting of a history of rheumatic heart disease, this patient is likely to have valvular aortic stenosis. Echocardiography is indicated for confirmation of the diagnosis and evaluation of the pressure gradient across the aortic valve. Echocardiogram demonstrates a thickened and calcified aortic valve with severe restriction of ~50 mmHg between the left ventricle and the aorta. There is concen- tric hypertrophy of the left ventricle without evidence of regional wall motion abnormalities. Given that the valve itself is markedly abnormal and there is already aortic regurgitation, balloon dilation is not likely to be effective. The surgeons must consider the patient’s size and interest in continued sports participation in their surgical planning. If this patient wants to continue sports participation, a valve that does not require life-long anticoagulation (Ross procedure or porcine valve) should be chosen. A 2-week-old infant is brought into the pediatrician’s office for a routine checkup. His birth history was unremarkable: the patient was born by normal, spontaneous vaginal delivery at 3. The patient’s mother reports that he was feeding well until 2 days ago, when he began to tire more quickly and fall asleep during feeds. On physical examination, the patient appears happy but tachypneic infant with mild subcostal retractions. Heart rate is 160, respiratory rate 50, and oxygen saturation in the right hand is 97%. There is a 2–3/6 systolic ejection murmur heard over the entire precordium with a gallop is present. There is mild hepatomegaly with the liver tip palpated at 4 cm below the costophrenic angle.

Bilateral segmental interstitial infiltrates may appear in 48 hours and are accompanied by severe hypoxemia buy discount ditropan 2.5mg line. However buy ditropan 2.5 mg visa, if influenza pneumonia A presents simultaneously with focal/segmental infiltrates and rapid cavitation in <72 hours order ditropan 2.5mg with visa, the likely pathogen is S. Avian influenza (H5N1) pneumonia and swine influenza (H1N1) pneumonia have not been complicated by simultaneous subsequent bacterial pneumonia. Therefore, the clinical history plus the appearance of cavitation points to the diagnosis, easily confirmed by Gram stain/culture of the sputum/blood. The patient’s history is important in identifying previously diagnosed disorders associated with specific immune defects. If severe pneumonia occurs during influenza season, then influenza is a likely diagnostic possibility. Because potential viral/fungal pathogens may be clinically indistinguishable, lung biopsy usually is needed for a specific diagnosis to determine optimal specific therapy. Immunosuppressed organ transplants presenting with bilateral symmetrical/interstitial infiltrates may be approached as those with mild/moderate hypoxemia versus those with severe hypoxemia. In cases without bacterial superinfection, prognosis is related to degree and duration of hypoxemia. In pandemic influenza A, as in 1918–1919, the majority of the deaths occurred in young, healthy adults without comorbidities and were due to severe hypoxemia uncompli- cated by bacterial pneumonia. During the past decade, avian influenza (H5N1) strains have circulated in Asia and Europe. Unlike influenza A, avian influenza (H5N1) is not efficiently transmitted from person-to-person, and for this reason does not, as yet have pandemic potential. However, in contrast to human influenza A, avian influenza (H5N1) is fatal in the majority of cases and affects primarily young healthy adults. Deaths from avian influenza (H5N1) occurs from severe hypoxemia uncomplicated by bacterial pneumonia. In the spring of 2009, the swine influenza (H1N1) pandemic began in Mexico and quickly spread throughout the world. Although large numbers of the population were affected by swine influenza (H1N1), there were relatively few mortalities. In the fatal cases of swine influenza (H1N1) pneumonia, like avian influenza (H5N1) pneumonia, fatalities died from severe hypoxemia also uncomplicated by bacterial pneumonia. The majority of fatalities with swine influenza (H1N1) pneumonia were young healthy adults without comorbidities (60–65). Optimal empiric therapy is based on correlating epidemiologic and clinical findings to arrive at a presumptive clinical diagnosis directed at the most likely pulmonary pathogen. Empiric therapy is continued until diagnostic possibilities are eliminated, and if possible, a specific etiologic diagnosis is made. Severe community-acquired pneumonia: determinants of severity and approach to therapy. Defining community-acquired pneumonia severity on presentation to hospital: an international derivation and validation study. Community-acquired pneumonia requiring admission to an intensive care unit: a descriptive study. Decreased mortality after implementation of a treatment guideline for community-acquired pneumonia. A five-year study of severe community acquired pneumonia with emphasis on prognosis in patients admitted to an intensive care unit. Impact of alcohol abuse in the etiology and severity of community-acquired pneumonia. Etiological diagnosis of community acquired pneumonia: utility of rapid microbiological methods with respect to disease severity. Patterns of resolution of chest radiograph abnormalities in adults hospitalized with severe community-acquired pneumonia. Empiric antibiotic therapy for community-acquired pneumonia: guidelines for the perplexed? Monotherapy versus dual therapy for community-acquired pneumonia in hospitalized patients. Effectiveness of early switch from intravenous to oral antibiotics in severe community acquired pneumonia: multicentre randomised trial. Severe community-acquired pneumonia due to Staphylococcus aureus, 2003–04 influenza season. Severe methicillin-resistant Staphylococcus aureus community-acquired pneumonia associated with influenza—Louisiana and Georgia, Decem- ber 2006-January 2007. Community-acquired methicillin-resistant Staphylococcus aureus carrying Panton-Valentine leukocidin genes: a lethal cause of pneumonia in an adult immunocompetent patient. Diagnostic and prognostic significance of relative lymphopenia in adult patients with influenza A. Delay in appropriate therapy of Legionella pneumonia associated with increased mortality. Seasonal influenza in Adults and Children–Diagnosis, Treatment, Chemoprophylaxis, and Institutional Outbreak Management: Clinical Practice Guidelines of the Infectious Diseases Society of America. Human Infection with Highly Pathogenic Avian Influenza A (H5N1) Virus: Review of Clinical Issues.