Case Presentation

A 3,600-g male term newborn, after birth, developed tachydyspnea and cyanosis. Delivery had been complicated by fetal nonprogression, and the umbilical cord was coiled twice around the neck and once around the chest. This caused hypoxemia and acute fetal suffering, as meconium emission during delivery showed.

Because of these symptoms, the infant was transferred to the neonatal intensive care unit, where continuous positive airway pressure ventilation with 50% fractional inspired oxygen and 4-cm water positive end-expiratory pressure was started. Chest radiography showed pulmonary venous congestion and patchy alveolar opacities ( Figure 1 ); arterial cord blood analysis showed severe acidosis, hypoxemia and hypercapnia. With continuous positive airway pressure ventilation, oxygen saturation was <70% and did not improve despite increasing to 100% fractional inspired oxygen. Therefore, invasive ventilation through orotracheal intubation was started, and 200 mg/kg of exogenous surfactant was instilled. Despite this treatment, there was neither clinical nor radiologic improvement, and oxygen saturation remained at about 85%.

Chest x-ray showing pulmonary venous congestion and patchy alveolar opacities.

Electrocardiography showed a prolonged corrected QT interval and T-wave inversion in leads I, aVL, V ₅ , and V ₆ ( Figure 2 ). Echocardiography ruled out a cardiac malformation but showed severe LVD because of the midapical akinesis of the anterior, posterior, and lateral walls as well as hyperkinesis of the ventricular septum (including the septal apex) and the basal segments of other walls ( Videos 1 and 2 ). LV ejection fraction was 30%. The left atrium was dilated, and there was severe functional mitral regurgitation ( Video 3 ). Two-dimensional and color Doppler examination showed normal origin and perfusion of the coronary arteries. The systolic velocity of trivial tricuspid regurgitation was 2.5 cm/sec.

Electrocardiogram showing a prolonged QT interval and T-wave inversion in leads I, aVL, V ₅ , and V ₆ .

Segmental wall motion abnormalities suggested an acute myocardial infarction or myocarditis. Serial troponin, serum glucose, and calcium levels were normal; infections were ruled out due not only to negative blood culture results but also to normal values for C-reactive protein and differential blood count.

At the same time, furosemide 1 mg/kg/day provided progressive clinical improvement by reducing the pulmonary congestion. After 7 hours of invasive ventilation, the newborn was extubated.

Echocardiography showed a progressive recovery of LV function and regression of mitral insufficiency ( Videos 4 and 5 ). The electrocardiographic abnormalities resolved within 10 days, and the newborn was discharged on day 11 without therapy, in good general condition. After 2 years, echocardiography confirmed normal LV function and the absence of mitral regurgitation.

Discussion

Perinatal complications may be associated with transient myocardial dysfunction due to hypoxia and/or ischemia.

In “stressed” newborns after either mild or merely suspected birth asphyxia, dysfunction of the right or left ventricle with respiratory problems of varying degrees of severity has also been reported. However, LVD has been described as global, not regional. Moreover, its pathogenesis has not been clarified.

In our case, the LVD involved segments of multiple epicardial coronary territories and was associated with hyperkinesis of the ventricular septum and basal segments of other walls. The LVD and mitral regurgitation contributed to respiratory distress and refractory hypoxemia.

Transient and regional LVD that does not correspond anatomically to coronary distribution suggests TS, correlated to psychophysical acute stress, including neurologic insults (subarachnoid hemorrhage, stroke, and seizure), respiratory distress (severe asthma exacerbation, airway obstruction, pneumothorax), metabolic insults (diabetic ketoacidosis), and catastrophic hemodynamic derangements (hypotensive gastrointestinal bleeding). TS appears to occur more often in postmenopausal women, but a few cases have also been reported in younger women and men. To the best of our knowledge, this is the first case reported in a newborn.

Presenting features typically include chest pain as well as electrocardiographic abnormalities, and heart failure can also occur. Electrocardiographic abnormalities include more prominent ST-segment elevation in leads V ₄ to V ₆ than in leads V ₁ to V ₃ , absent reciprocal changes in the inferior leads, precordial or global T-wave inversion, and prolongation of the QT interval. However, electrocardiographic results can be normal or can show either nonspecific T-wave abnormalities or major ST elevation across the precordial and limb leads. In our case, electrocardiography showed T-wave inversion in leads I, aVL, V ₅ , and V ₆ and a prolonged corrected QT interval ( Figure 2 ).

The classic appearance of TS seen initially on contrast angiography is characterized by akinesis of the mid and apical segments and hyperkinesis of the basal portion of the left ventricle, producing a pattern of “apical ballooning” and resembling a traditional Japanese octopus trap, or takotsubo, with a round bottom and narrow neck. Apical ballooning is the usual pattern, but several recent reports have described TS associated with the suppression of basal contraction and apical sparing or with the ballooning of the midventricle only. Therefore, varying patterns of LV wall motion abnormalities are possible in TS, with transient LV apical ballooning being more common and other regional wall motion abnormalities less common.

This case is not the typical apical ballooning syndrome, because of hyperkinesis of the mid and apical segments of the ventricular septum; rather, it is a different form of stress cardiomyopathy. Nevertheless, the diagnostic criteria for TS (wall motion abnormalities in the LV mid segments with or without apical involvement and extending beyond a single epicardial coronary distribution) were fulfilled in our case.

This syndrome can be complicated by mitral regurgitation due to systolic anterior motion of the anterior mitral leaflet. In our patient, mitral regurgitation was caused by LV dilation that prevented adequate coaptation of anatomically normal leaflets as occurs in functional mitral regurgitation.

What determines regional LVD is difficult to establish. Among the possible causes, we consider delivery stress, with excessive sympathetic stimulation and catecholamine-mediated cardiac toxicity. Wittstein et al. highlighted the effects that stress can have on myocardial contractility. Catecholamines might determine a direct toxic effect on cardiac myocytes. Indeed, the mechanism could be a decrease in myocyte viability through calcium overload mediated by cyclic adenosine monophosphate. The segmental systolic dysfunction may reflect regional myocardial differences in responsiveness to sympathetic stimulation or catecholamine sensitivity.