Congenital Pulmonary Stenosis - Erler Zimmer 3D anatomy Series MP2034

This dissection model highlighting Congenital Pulmonary Stenosis is part of the exclusive Monash 3D anatomy series, a comprehensive series of human dissections reproduced with ultra-high resolution color 3D printing.

Clinical History.

This male child had a heart murmur discovered at birth. He remained healthy until the age of 10 months. Two weeks before hospitalization, he became languid, developed a mild cough, and suddenly gained a lot of weight. Ten days before admission, he developed swelling in his hands, feet and face. On examination he was a chubby baby with a blood pressure of 90/59 mmHg. There was a good shivering all over the precordium, and a maximal systolic murmur in the lung area. There was edema of the face and legs. After admission, the child gained additional weight, and there was no response to therapy. Death was caused by congestive heart failure.

Pathology

The specimen is the child's heart. View from the left side and note that the pulmonary artery was opened to visualize the upper surface of the pulmonary valve. This abnormal valve consists of a thickened conical diaphragm with a 2 mm diameter opening at the apex. The open pulmonary artery has a large post-stenotic dilatation. There is right heart enlargement due to marked dilatation of the right atrium and right auricle (open) and hypertrophy of the right ventricle (the wall has been cut in two places [one penetrating, one not] to expose the hypertrophied myocardium). This is pure pulmonary valve stenosis.

Further information

Pulmonary stenosis accounts for about 7% of congenital heart disease. However, it can also occur later in life as a result of increased pulmonary pressures. In infants, it can occur as an isolated defect or as part of a more complex lesion, such as tetralogy of Fallot.
The latter is a combination of four congenital anomalies, including a ventricular septal defect, pulmonary valve stenosis, misplaced aorta, and right ventricular hypertrophy. Abnormalities similar to this specimen can now be corrected surgically.
The natural course of congenital pulmonary stenosis varies with age and severity at the age of development. Pulmonary stenosis can be considered on a scale of mild, moderate, and severe. While mild pulmonary stenosis can remain asymptomatic and indolent without diagnosis for several years, moderate pulmonary stenosis can progress rapidly to severe stenosis. Severe pulmonary stenosis has significant effects on the heart due to increased pressures leading to right ventricular hypertrophy. Severe pulmonary stenosis is often associated with right ventricular outflow obstruction and subsequent heart failure. Therefore, moderate to severe pulmonary stenosis may manifest as dyspnea, chest pain, or syncope.

Other complications of severe pulmonary stenosis include infective endocarditis and arrhythmias caused by remodeling and scarring of the ventricular and atrial walls. The diagnosis of pulmonary stenosis is often obtained by transthoracic echocardiography. Other imaging modalities include MRI and CT.

What advantages does the Monash University anatomical dissection collection offer over plastic models or plastinated human specimens?

• Each body replica has been carefully created from selected patient X-ray data or human cadaver specimens selected by a highly trained team of anatomists at the Monash University Center for Human Anatomy Education to illustrate a range of clinically important areas of anatomy with a quality and fidelity that cannot be achieved with conventional anatomical models-this is real anatomy, not stylized anatomy.
• Each body replica has been rigorously checked by a team of highly trained anatomists at the Center for Human Anatomy Education, Monash University, to ensure the anatomical accuracy of the final product.
• The body replicas are not real human tissue and therefore not subject to any barriers of transportation, import, or use in educational facilities that do not hold an anatomy license. The Monash 3D Anatomy dissection series avoids these and other ethical issues that are raised when dealing with plastinated human remains.