Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with other types of CHD (such as heterotaxy or HLHS). TAPVR is defined as an abnormal connection where all pulmonary veins do not drain into the left atrium but into the right atrium either directly or through a vein that is connected to the right atrium. TAPVR can be divided into four anatomic groups (Fig. 32.1): (1) supracardiac (about 55%), (2) cardiac (about 30%), (3) infracardiac (about 13%), and (4) mixed (very rare). In addition, it can be divided into two physiological types: nonobstructed and obstructed. Embryologically, all pulmonary veins usually connect to a pulmonary venous confluence that connects to the left atrium. If this connection does not occur, the pulmonary venous confluence connects to a systemic vein instead.

暂无摘要。

A 59-year-old Japanese woman was admitted with heart failure due to severe pulmonary regurgitation and tricuspid regurgitation, in addition to atrial fibrillation 45 years after surgical correction of tetralogy of Fallot (TOF). She had been under treatment with medication and catheter ablation for arrhythmia including ventricular tachycardia for the past 28 years. She underwent pulmonary valve replacement as well as tricuspid and mitral valvuloplasty, which obviously improved her status even though her right ventricular end-diastolic volume index exceeded the recommended threshold. Patients who have undergone surgical correction of TOF need to be managed over the long term. .

A 73-year-old Japanese man was admitted with an asymptomatic pulmonary artery aneurysm. However, chest X-ray and contrast-enhanced thoracic computed tomography revealed a protrusion at the second left branch that in fact was a pulmonary artery aneurysm with a diameter of 50 mm. Transesophageal echocardiography showed a bicuspid pulmonary valve, and cardiac catheterization revealed pulmonary stenosis with a pressure gradient of 45 mmHg, but no other heart diseases were noted. An extremely rare pulmonary artery aneurysm associated with an isolated bicuspid pulmonary valve was diagnosed.

BACKGROUND: This case study\'s novelty lies in the potential to link a new sign in pulmonary embolism diagnosis which does not increase cost but could lead to more rapid treatment. Early intervention in these cases is vital to decrease morbidity and mortality.
METHODS: An otherwise healthy 20-year-old female patient presents to the emergency department for evaluation of a syncopal episode which occurred just prior to arriving to the emergency department. Patient also complains of ongoing shortness of breath while performing activities of daily living for 3 weeks. In this patient with no known valvular disease, physical exam revealed a systolic murmur heard only posteriorly. Subsequent emergency department workup revealed bilateral massive pulmonary emboli.
CONCLUSIONS: A new flow murmur heard in atypical locations could be an early sign to aid in the detection and diagnosis of pulmonary embolism. This is especially important in rural community hospitals with limited access to advanced imaging modalities.

暂无摘要。

Acute pulmonary embolism with significant right ventricular strain could be associated with a low-pitch peripheral systolic murmur radiating to the axillae.

A 40-year-old male patient presented with dyspnea on exertion and palpitation from 2 months ago. Physical examination revealed normal vital sign and a systolic murmur grade 3/6 in aortic area. Echocardiography revealed unicuspid aortic valve with an eccentric orifice, calcification, and aortic valve area of 0.9 cm2. This case report highlights the usefulness of three-dimensional echocardiography for the determination of number of aortic valve cusps, presence of raphe and morphology of valve.

暂无摘要。

Ventricular septal defect (VSD) has a relatively non-aggressive clinical course; either spontaneous closure or causing congestive heart failure treatable with surgical intervention. We present two autopsy cases of sudden infant deaths from clinically undiagnosed VSDs. Case 1 was an 18-day-old boy. As the deceased coughed and became limp after feeding, he was carried to a hospital. Heart murmur was not auscultated there, so he was brought back to home. He presented severe dyspnea and then he was pronounced dead the next day. Case 2 was a 3-week-old boy. Any abnormality was pointed out at physical examination, although his mother felt his wheeze. He developed respiratory arrest at home in the morning and then he was confirmed dead at the hospital. Heart weights of these babies were heavier than mean weights of each normal development. There were perimembranous VSDs in both cases. Histology revealed that the pulmonary arterial walls were thickened. We diagnosed the cause of death in these cases was cardiac collapse with pulmonary hypertension due to VSD. Congenital heart diseases can be diagnosed as early as before birth, because echocardiograph and fetal echography are prevalent in these days. Most VSDs can be noticed by systolic murmur even today. We consider that the failure of initial clinical diagnosis of VSD in primary physical assessment could lead unexpected sudden death. These two cases reminded us to the importance of auscultation which is conventional but as one of the indispensable measure to find a clue for the congenital abnormality.