Extrinsic compression of the left main coronary artery (LMC) by the pulmonary artery (PA) is a very unusual and poorly understood entity, usually associated with the presence of adult congenital heart disease. We identified 12 patients (age range, 6 months to 55 years) with LMC stenosis (> or = 50%) presumably secondary to compression by a dilated main PA and related to various forms of heart disease (11 congenital, 1 pulmonary hypertension). In all cases, the main PA was dilated with the main PA/aortic root diameter increased (mean, 2.0; normal value, < or = 1.0), and in all but two, PA pressures were increased (> 30 mm Hg systolic). Left coronary trunk stenosis was usually visualized in only one angiographic view (best seen in 45 degrees left anterior oblique, 30 degrees cranial projection). The LMC also appeared to be inferiorly displaced and in close contact with the left aortic sinus (mean angle between sinus and LMC was 23 degrees +/- 13 degrees, a control group was 70 degrees +/- 15 degrees ). In one patient, surgical correction of the dilated PA was associated with a reduction in LMC stenosis from 85% to < 50% and less inferior left main displacement (from 25 degrees to 50 degrees ). Patients with a dilated main PA may exhibit extrinsic LMC compression leading to significant eccentric narrowing and downward displacement of the LMC. In the presence of significant dilatation of the main PA from any etiology, functional and/or anatomic studies should be performed to exclude significant LM obstruction.

Multiple radiofrequency pulses applied inside the pulmonary veins ostia can induce severe pulmonary venous stenosis and veno-occlusive pulmonary syndrome.

Fetal echocardiography has recently caused an impact on the treatment of congenital heart disease and in the field of therapeutic, cardiological intervention. The present study reports on a case of critical aortic stenosis, diagnosed in utero at 27 weeks’ gestation, and in which balloon dilatation was attempted to improve the poor prognosis associated with this condition. Since the endocardium at this stage of development was apparently normal, this therapeutic intervention was attempted to avoid irreversible damage to the left ventricle. Although hydrops disappeared and the myocardium hypertrophied, endocardial fibroelastosis progressed and the neonate died within the first day of life, after surgical aortic valvotomy. More data are necessary to clarify whether endocardial fibroelastosis is really a consequence of high pressure in the left ventricle resulting from stenosis of the aortic valve or whether it is a disease, the progression of which is unavoidable once it takes hold.

Management of pediatric pulmonary hypertension associated with congenital heart disease (PHT-CHD) is challenging. Some patients have persistently elevated pulmonary artery pressure (PAP) after cardiac surgery, an undesired condition that is difficult to predict. We investigated the value of clinical, hemodynamic, and histopathological data in predicting the outcome in a prospective cohort. Patients with PHT-CHD received sildenafil orally pre- and postoperatively for six months and then were subjected to a catheter study. Thirty-three patients were enrolled (age range = 4.6–37.0 months). Pulmonary vascular resistance (PVR) was 4.9 (range = 3.9–7.2) Wood units × m2 (median with IQR). Twenty-two patients had a ≥ 20% decrease in PVR and pulmonary-to-systemic vascular resistance ratio (PVR/SVR) in response to inhaled nitric oxide (NO). The response was directly related to the degree of medial hypertrophy of pulmonary arterioles ( P < 0.05) (morphometric analysis, intraoperative lung biopsy). Subsequently, five of the non-responders had a ≥ 30% increase in pulmonary blood flow in response to sildenafil (3.0 [2.0–4.0] mg/kg/day). Six months after surgery, PAP and PVR were significantly lower ( P < 0.001 vs. baseline), even in seven patients with Heath-Edwards grade III/IV pulmonary vascular lesions ( P = 0.018), but still abnormal in 12 individuals (>25 mmHg and >3.0 U × m2, respectively). A preoperative PVR/SVR of ≥24% during NO inhalation and a wall thickness of arteries accompanying respiratory bronchioli of ≥4.7 (Z score) were identified, respectively, as risk and protection factors for abnormal postoperative hemodynamics (hazard ratio [95% CI] = 1.09 [1.01–1.18], P = 0.036; and 0.69 [0.49–0.98], P = 0.040, respectively). Thus, in PHT-CHD patients receiving oral sildenafil pre- and post-surgical repair of cardiac lesions, mid-term postoperative outcome is predictable to some extent.

The purpose of this study was to evaluate the hemodynamic effects of inhaled nitric oxide in oxygen (NO + O2) in patients with pulmonary hypertension. Eighteen patients (median age 31.5 months) with pulmonary hypertension inhaled through a mask 100% O2 and 20 parts per million NO + inspired O2 fraction (FiO2) at 0.4. Hemodynamic measurements were made at baseline and after O2 and NO + O2 administration. The pulmonary vascular resistance index decreased after inhalation of O2 and NO + O2 (p = 0.0018 and p = 0.0003, respectively), the decrease being significantly greater after NO + O2 (p = 0.0311). Concerning the transpulmonary pressure gradient, a reduction occurred in values after O2 and NO + O2 inhalation when compared with baseline values (p = 0.0014 and p = 0.0008). In patients with congenital heart disease, an increase occurred in pulmonary blood flow after O2 (p = 0.0089) and NO + O2 (p = 0.0019) compared with baseline values, and an increase also occurred in the pulmonary/systemic blood flow ratio after NO + O2 (p = 0.0017). The main side effect related to NO + O2 was pulmonary congestion in 3 patients. Low doses of NO combined with O2 demonstrated a selective pulmonary vasodilator response in patients with pulmonary hypertension. Despite its use for testing pulmonary reactivity, inhalation of NO + O2 should be carefully administered because of the potential risk of pulmonary congestion.

Coronary artery anomalies are congenital changes in their origin, course, and/or structure. Most of them are discovered as incidental findings during coronary angiographic studies or at autopsies. We present herein the case of a 70-year-old man with symptomatic severe aortic valvar stenosis whose preoperative coronary angiogram revealed a so far unreported coronary anomaly circulation pattern.

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Reference diameter of coronary vessels at the site of lesions treated by stenting is significantly influenced by a variety of characteristics. We hypothesize that the treated segment size of patients undergoing stenting ultimately reflects the conjoint effect of several different factors, including constitutional, anatomical, and clinical features.